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The First Asia-Pacific Congress of Pediatric Cardiology and Cardiac Surgery November 1-4, 2006 Bangkok Convention Center (BCC) Sofitel Central Plaza Bangkok, Thailand Program and Abstracts Welcome Message Message from Chairman Organizing Committee It gives me great pleasure to welcome you to The First Asia-Pacific Congress of Pediatric Cardiology and Cardiac Surgery which is held during November 1-4, 2006 at Bangkok Convention Center, Sofitel Central Plaza, Bangkok, Thailand. This year is a very important year for Thai people to cerebrate the Sixtieth Anniversary Cerebration of His Majesty’s Accession to the throne. Asian Pacific Pediatric Cardiology Society was founded in 1979. Scientific meeting was a one day program during Asia-Pacific Congress of Cardiology. With a rapid progress in the area of pediatric cardiology, cardiac surgery and related fields, president of national societies agreed to hold a separate meeting every two years. We are proud to host this first meeting which was originally planned to be in Shanghai. Our Scientific Committee has been working very hard with time frame of less than one year trying to make this meeting beneficial to all of us. We have invited world renowned experts as well as experts in Asia-Pacific in all area related to the care of children with heart diseases with emphasis on problems in developing countries in Asia-Pacific and recent advance knowledge and technology. In addition, this congress will provide a wonderful opportunity for discussion and sharing experiences for future co-operation. On behalf of the Organizing Committee, I wish your staying in Thailand is an enjoyable one. Boonchob Pongpanich, M.D., F.A.C.C. Chairman Organizing Committee Welcome Message Dear all, On behalf of the Asia-Pacific Pediatric Cardiac Society ( APPCS ) it gives me immense pleasure to welcome you to the First Biannual Conference of the Society to be hosted by Thailand in Bangkok. As you are aware the Asia-Pacific society of Pediatric cardiology was renamed the APPCS at the World Congress in Beunos Aires with a view to encourage equal participation from cardiologists, surgeons, intensivists, anesthesiologists, pediatricians, radiologists and others involved in the care of children with heart disease. It was also recognized that the current practice of having a pediatric sub-section at the biannual meeting of the Asia-Pacific Society of Cardiology was proving inadequate and that ample advances have occurred in the field of pediatric cardiac care in this region to merit a separate conference. Additionally, problems and practices in pediatric cardiac care in the Asia Pacific region differ significantly from those in the Americas and Europe. It is time that we have a forum where we can deliberate upon and find solutions to our regional problems. I would like to thank our colleagues from Thailand who have readily volunteered to take on the challenge and host the first conference at short notice. As members of the society we should provide them with all the help and support that they need to make the conference a grand success and I urge all of you to make every effort to attend the meeting. I also request you to pass on this information to all your colleagues involved in pediatric cardiac care, as we are still in the early days of the new society and our database is still being compiled. Please feel free to respond with suggestions and comments. I look forward to seeing you all in Bangkok. Krishna Iyer, M. Ch. President, Asia-Pacific Pediatric Cardiac Society Director, Pediatric & Congenital Heart Surgery, Escorts Heart Institute & Research Centre, Okhla Road, New Delhi, INDIA 110025 Welcome Message Dear friends, On behalf of The Society of Pediatric Cardiology of Thailand, I would like to extend all of you a very warm welcome to Thailand, the land of smile, to participate in the First Asia-Pacific Congress of Pediatric Cardiology and Cardiac Surgery to be held in Bangkok during November 1-4, 2006. Besides seeing old friends and making new friends, gaining new knowledge in the field of Pediatric Cardiology, Cardiac Surgery and related fields from all the specialists invited to this Congress, I hope you would find opportunities to see our beautiful country and taste our famous food. Table Last but not least, I hope some of you would be able to extend your stay in Bangkok after the Congress to enjoy our Loy Krathong festival. I wish this Congress a very successful one and hope that it would be one of the most memorable ones for all of us. Have a very good time in Thailand, and … Sawasdee Professor Emeritus Chule Thisyakorn, M.D. President, The Society of Pediatric Cardiology of Thailand Welcome Message Dear Friends and Colleagues, It is our honor to host the First Asia-Pacific Congress of Pediatric Cardiology and Cardiac Surgery in Bangkok, Thailand. This is the first scientific meeting of the Asia-Pacific Pediatric Cardiac Society which was previously known as the Asian-Pacific Society of Pediatric Cardiology. Our previous scientific meetings were done in conjunction with the AsiaPacific Society of Cardiology. Through these years, we have seen the progress and healthy growth and maturation of the pediatric cardiology and cardiac surgery in Asia-Pacific to warrant its own scientific meeting. The First Congress in Thailand cannot be success without the tremendous help from our friends and colleagues. In addition to many invited outstanding world renowned cardilogists, surgeons and investigators, our Asia-Pacific speakers will also present their unique knowledge and experience. The AsiaPacific Congress of Pediatric Cardiology and Cardiac Surgery will aim to provide a forum for discussion of a broad spectrum of topics ranging from issues concerning countries in this region with emerging economies to the state-of-the-art knowledge and break-through technologies. Participants will have the opportunity to acquaint themselves with some of the latest technical innovations in each field. Most importantly, this will help to build up the capacity of the participants for an improvement of care and treatment for children with heart diseases. Furthermore, the congress has served the occasion for renewal and further strengthening of our friendship, which already exists within Asia-Pacific and with international colleagues. On behalf of the scientific committee, I would like to extend my warm welcome to all our friends and delegates from Asia-Pacific and other countries and wish you a very pleasant and memorable stay in Thailand. Please take advantage of your time to explore the beautiful country of Thailand as well as to enjoy the exciting city of Bangkok. Warmest regards, Apichai Khongphatthanayothin, M.D. Kritvikrom Durongpisitkul, M.D. Co-Chairman Scientific Chairman, Chaisit Sangtaweesin, M.D. Co-Chairman Pirapat Mokarapong, M.D. Secretary General Table of Contents Welcome Message Chairman Organizing Committee President, Asia-Pacific Pediatric Cardiac Society President, The Society of Pediatric Cardiology of Thailand Scientific Chairman Organizing Committee Advisory Committee Scientific Committee Asia-Pacific Pediatric Cardiac Society (APPCS) Invited International Speakers Invited Asia-Pacific Speakers General Information Registration Information Guidelines for Speaker’s Presentation Instruction for Poster Presentation Program at a Glance Scientific Program Tuesday, October 31, 2006 Wednesday, November 1, 2006 Lunch Symposium Thursday, November 2, 2006 Lunch Symposium Friday, November 3, 2006 Lunch Symposium Saturday, November 4, 2006 Speaker’s Abstracts (November 1, 2006) Speaker’s Abstracts (November 2, 2006) Speaker’s Abstracts (November 3, 2006) Speaker’s Abstracts (November 4, 2006) Abstract for Free Paper Free Paper 1-2 Free Paper 3-4 Free Paper 5-6 Free Paper 7-8 Free Paper 9, Young Investigator Award Table of Contents Abstract for Poster Instruction for Poster Presentation Poster Group 1: PS1-PS 24 Poster Group 1: PS25-PS 48 Poster Group 1: PS49-PS 70 Poster Group 1: PS71-PS 94 Poster Group 1: PS95-PS 119 Map Of BTS & MRTA in Bangkok Floor Plan, Bangkok Convention Center 4th Floor 5th Floor Trade Exhibitors Social Program Index of Abstracts Index of Authors Acknowledgements Organizing Committee Honorary Advisors: - Chairman: Vice Chairman: Secretary General: Scientific Coordinator: Treasurer: Social: Audio Visual: Publication: Congress Facility & Exhibition: Executive Committee: Chadsri Prachuabmoh, M.D. Charlie Phornphatkul, M.D. Choompol Vongprateep, M.D. Chotima Pathamanand, M.D. Pantpis Sakornpant, M.D. Santa Ha-Upala, M.D. Suphachai Chaithiraphan, M.D. Usa Thisyakorn, M.D. Virojna Sueblinvong, M.D. Boonchob Pongpanich, M.D. Chule Thisyakorn, M.D. Pirapat Mokarapong, M.D. Suthep Wanitkun, M.D. Worakan Promphan, M.D. Apichai Khongphatthanayothin, M.D. Kritvikrom Durongpisitkul, M.D. Chaisit Sangtaweesin, M.D. Jarunun Ungbhakorn Sureeporn Kunathai, M.D. Chantana Daorai Pimjai Bodhipakti Teerachat Silarat, M.D. Chalermkiet Tuntrakool, M.D. Vichai Benjacholamas, M.D. Pornthep Lertsapcharoen, M.D. Jurapim Soongswang, M.D. Boonchu Sririchongkolthong, M.D. Pirapat Mokarapong, M.D. Tawatchai Kirawittaya, M.D. Worakan Promphan, M.D. Apichart Nana, M.D. Chareonkiat Roekkliang, M.D. Cherdchai Tontisirin, M.D. Manat Panamonta, M.D. Oranuch Kyokong, M.D. Pongsak Khowsathit, M.D. Somchai Sriyoschati, M.D. Somkiat Sopontammarak, M.D. Sukasom Attanawanich, M.D. Supachai Chuerattanaphong, M.D. Supreecha Tanamai, M.D. Ungkab Prakanrattana, M.D. Yupada Pongprot, M.D. Advisory Committee Anna U Rahayoe, M.D. Antony C Chang, M.D. Edgardo E. Ortiz, M.D. Gary Sholler, M.D. Heung Jae Lee, M.D. Hung Chi Lue, M.D. In Sook Park, M.D. Jim Wilkinson, M.D. Jinfen Liu, M.D. Kalim Uddin Aziz, M.D. Koichiro Niwa, M.D. Krishna Iyer, M.D. Krishna Kumar, M.D. Lee Chuen Neng, M.D. Lee Sang Bum, M.D. Makoto Nakazawa, M.D. Masood Sadiq, M.D. Maurice Leung, M.D. Mazeni Alwi, M.D. Mei Hwan Wu, M.D. Shunji Sano, M.D. Sukman Putra, M.D. Teiji Akagi, M.D. William C Yip, M.D. (Indonesia) (USA) (Philippines) (Australia) (Korea) (Taiwan) (Korea) (Australia) (China) (Pakistan) (Japan) (India) (India) (Singapore) (Korea) (Japan) (Pakistan) (Hong Kong) (Malaysia) (Taiwan) (Japan) (Indonesia) (Japan) (Singapore) Scientific Committee General Cardiology: Duangmanee Laohaprasittiporn, M.D. Thanarat Layangool, M.D. Rattanaporn Pornkul, M.D. Suthep Wanitkun, M.D. Kritvikrom Durongpisitkul, M.D. Pornthep Lertsapcharoen, M.D. Apichai Khongphatthanayothin, M.D. Preecha Laohakunakorn, M.D. Vichai Benjacholamas, M.D. Vorasin Ketanon, M.D. Vichao Kojuranjit, M.D. Samphant Pornvilawan, M.D. Thivakorn Kasemsri, M.D. Dusit Staworn, M.D. Satid Thammasitboon, M.D. Yuttapong Wongswadiwat, M.D. Worakan Promphan, M.D. Vachara Jamjureeruk, M.D. Jarupim Soongswang, M.D. Aungkana Gengsakul, M.D. Anant Khositseth, M.D. Alisa Limsuwan, M.D. Rutja Phuphaibul, R.N., Ph.D. Echo & Non – Invasive Imaging: Intervention Cardiology: Arrhythmia & Electrophysiology: Surgery: Intensive Care: Genetic & Fetal Cardiology: Adult Congenital Heart Disease: Nursing: Asia-Pacific Pediatric Cardiac Society Asia-Pacific Pediatric Cardiac Society Interim Officer 2005-2006 President: Krishna Iyer, M.D. (India) Secretary: Apichai Khongphatthanayothin, M.D. (Thailand) Steering Committee* Country Australia Steering Committee Gary Sholler, M.D. Dan Penny, M.D. China Hong Kong Leung Ping, Maurice, M.D. India Krishna Iyer, M.D. R. Juneja, M.D. Indonesia Anna U. Rahayoe, M.D. Sukman Putra, M.D. Japan Makoto Nakazawa, M.D. Koichiro Niwa, M.D Korea Heung Jae Lee, M.D. Lee Sang Bum, M.D. Malaysia Geetha Kandavello, M.D. Mazeni Alwi, M.D. Pakistan Kalimudin Aziz, M.D. Masood Sadiq, M.D. Philippines Rhodora de Leon, M.D. Della Pelaez, M.D. Singapore William Yip, M.D. Quek Swee Chye, M.D. Sri Lanka Sepalika Mendis, M.D. Taiwan Mei-Hwan Wu, M.D. Jou-Kou Wang, M.D. Thailand Boonchob Pongpanich, M.D. Apichai Khongphatthanayothin, M.D. Vietnam Nguyen Lan Hieu, M.D. *From countries that were represented at the Business Meeting of the APSPC in Buenos Aires, Argentina, Septermber 2005. Invited International Speakers Afksendiyos Kalangos, M.D. Clinique de Chirurgie Cardio Vasculaire, HCUG, Geneva, Switzerland Andrew N. Redington, M.D. Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada Andrew Cook, M.D. Cardiac Unit, Institute of Child Health, University College London, UK Anthony C. Chang, M.D., MBA CHOC Heart Institute, Children’s Hospital of Orange County, Orange, CA, USA Bernadette McCormick, R.N., C.C.R.N. University of California, San Diego, San Diego, California, USA Christof Stamm, M.D. Deutsches Herzzentrum Berlin Augustenburger Platz 1, Berlin, Germany David J. Sahn, M.D. Professor of Pediatrics, Diagnostic Radiology, Obstetrics & Gynecology and Biomedical Engineering Director, OHSU Interdisciplinary Program in Cardiac Imaging, Pediatric Cardiology, Oregon Health & Science University, Portland, OR, USA Laurance L. Lequier M.D., FRCP(C) Director, ECLS Program, Stollery Children’s Hospital, Canada Michael J. Silka, M.D. Division of Cardiology, Childrens Hospital Los Angeles, University of Southern California, Los Angeles, California, USA Michael A. Gatzoulis, M.D., Ph.D., FACC, FESC Professor of Cardiology, Congenital Heart Disease and Consultant Cardiologist, Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College, London, UK Roger B. B. Mee, M.D. Chairman Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic, USA (Retired) Thomas L. Spray, M.D. Children’s Hospital of Philadelphia, PA, USA Ziyad M. Hijazi, M.D., MPH, FACC, FAAP, FSCAI George M. Eisenberg, Professor of Pediatrics and Medicine, Chief, Section of Pediatric Cardiology, Medical Director, The University of Chicago Heart Center, University of Chicago, IL USA Invited Asia-Pacific Speakers Australia Dan Penny, M.D., Ph.D., FRACP Director of Cardiology, The Royal Children’s Hospital, Professorial Fellow, Dept. of Paediatrics, University of Melbourne, Australia Geoffrey K Lane, B Pharm, MB.BS, FRACP Interventional Cardiologist, Royal Children’s Hospital, Honorary Cardiologist, Royal Melbourne Hospital, Melbourne, Australia James L Wilkinson, M.D. Honorary Cardiologist, Royal Melbourne Hospital, Melbourne, Australia Samuel Menahem, M.D. Department of Paediatric Cardiology, Monash Medical Centre, Victoria Australia. China Guo-Ying Huang, M.D. Professor of Pediatrics, Director of Echo-Cardiology Lab, Pediatric Heart Center, Executive Vice President, Children’s Hospital of Fudan University, Shanghai, PR China Jinfen Liu, M.D. Chief Physician, Shanghai Children Medical Center, Shanghai, China Lily Lihwa Hsu, RN., MSN Program Director Nursing Education-Shanghai, Deputy Dean Shanghai Jiao Tong University School of Nursing, Preject HOPE Shanghai Office, Shanghai Jiao Tong University School of Medicine, Shanghai, China Yonghao Gui, M.D., MSc President , Children’s Hospital of Fudan University, Vice Chairman, Chinese Pediatric Society, Shanghai, China Zhuoming XU, M.D. Shanghai Children Medical Center, Shanghai China Zhiwei Xu, M.D. Shanghai Children Medical Center, Shanghai China Li Juan Fu Vice Dean School of Nursing Shianghai, Jiao Tong University, Shanghai China Germany Trong-Phi Le, M.D. Department of Pediatric Cardiology, University of Hamburg, Germany Invited Asia-Pacific Speakers India Amit Varma, M.D. Director, Medical Services & Business Planning, Director Critical Care, New Delhi, India Krishna Iyer, M.D. Director, Pediatric & Congenital Heart Surgery, Escorts Heart Institute and Research Center, New Delhi, India Neeti Chandra, M.D. Fortis Healthcare Ltd., New Delhi, India Pavathi Iyer, M.D. Pediatric & Congenital Heart Surgery, Escorts Heart Institute and Research Center, New Delhi, India Sunita Maheshwari, M.D. Consultant Pediatric Cardiologist, Narayana Hrudayalaya, Bangalore, India Indonesia Anna U. Rahayoe, M.D. President, National Cardiovascular Center, Jakarta, Indonesia Najib Advani, M.D. MMed Consultant Pediatric Cardiologist, University of Indonesia, Jakarta, Indonesia Sukman T Putra, M.D. FACC, FESC Consultant Pediatric Cardiologist, Division Chief, Pediatric Cardiology, Department of Pediatric, University of Indonesia, Cipto Mangunkusumo General Hospital, Jakarta, Indonesia Japan Hiromi Kurosawa, M.D. Department of Cardiovascular Surgery, Tokyo Women’s Medical University, Tokyo, Japan Katuhino Tatsuno, M.D. President, Chiba Cardiovascular Center, Chiba, Japan Koichiro Niwa M.D. Ph.D. FACC Chief, Department of Pediatrics, Director, Adult Congenital Heart Disease Program, Chiba Cardiovascular Center, Japan Invited Asia-Pacific Speakers Makoto Nakazawa, M.D. Director, Pediatric and Lifelong Congenital Cardiology Institute, Southern TOHOKU General Hospital, Southern TOHOKU Research Institute, Fukushima, Japan Masao Yoshinaga, M.D. Head, Dept of Pediatrics, National Hospital Organization, Kagoshima Cardiovascular & Cancer Center Kagoshima, Japan Shunji Sano M.D., Ph.D. Professor and Chairman, Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan Teiji Akagi, M.D. Cardiac Care Unit, Okayama University Hospital, Okayama, Japan Yasuki Maeno, M.D. Maternal and Perinatal Medical Center, Department of Pediatrics and Child Health, Kurume University School of Medicine, Kurume, Japan Yoshihide Mitani, M.D., Ph.D. Department of Pediatrics, Mie University School of Medicine, Japan Korea Dong Man Seo, M.D. Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Eun Jung Bae, M.D., Ph.D. Associate Professor, Department of Pediatrics, Seoul National University College of Medicine, Seoul, South Korea Heung Jae Lee, M.D. Professor of Pediatrics, Sungkyunkwan University School of Medicine, Cardiac & Vascular Center, Samsung Medical Center, Seoul, Korea Hyun Woo Goo, M.D. Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea In-Sook Park, M.D. Dean, Professor in Pediatric Cardiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Sang Bum Lee, M.D. Kyungpook National University, School of Medicine, Department of Pediatrics, Taegu, Korea Invited Asia-Pacific Speakers Seong-Ho Kim, M.D., Ph.D. Director, Cardiovascular Center, Chairman, Department of Pediatrics, Staff Pediatric Cardiologist Halla General Hospital, South Korea Malaysia Geetha Kandavello, M.D. Consultant Pediatric Cardiologist, Dept of Pediatric Cardiology, Institut Jantung Negara, National Heart Institute, Kuala Lumpur, Malaysia Mazeni Alwi, M.D. Consultant & Head, Department of Paediatric Cardiology, Institut Jantung Negara (National Heart Institute), Kuala Lumpur, Malaysia Mohamed Azhari Yakub, M.D. National Heart Institute, Dept of Cardiothoracic Surgery, Kuala Lumpur, Malaysia New Zealand Nigel Wilson, M.D. Pediatric Cardiologist, Green Lane Hospital Clinical Services, Starship Hospital, Auckland, New Zealand Pakistan Kalim Uddin Aziz, M.D. Professor (Emiritus) of Paediatric Cardiology, National Institute of Cardiovascular Diseases, Pakistan Masood Sadiq, M.D. Professor of Pediatric Cardiology, The Children’s Hospital/The Institute of Child Health and The Punjab Institute of Cardiology, Lahore, Pakistan Philippines Edgardo E. Ortiz, M.D. Chairperson and Professor of the Department of Pediatrics, UP-PGH, College of Medicine-Philippine General Hospital, University of the Philippine, Manila, Philippines. Luis Mabilangan, M.D. Professor Emeritus, University of Philippines College of Medicine, Manila, Philippines Invited Asia-Pacific Speakers Saudi Arabia Mohammed Omar Galal, M.D. Cardiovascular Department, King Faisal Specialist Hospital and RC, Jeddah, Saudi Arabia. Singapore James Yip, M.D., MRCP Director, Adult Congenital Heart Programme, The Heart Institute, National University Hospital, Singapore. William C.L. Yip, M.D. National University Hospital, Singapore Baby & Child Clinic, Gleneagles Medical Centre, Singapore Taiwan Hung Chi Lue, M.D. President and Physician-in-Chief, MS General Hospital, Professor Emeritus of Pediatrics, National Taiwan University, Taiwan Chun-Wei Lu, M.D. Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan Jou-Kou Wang, M.D. National Taiwan University, Division of Pediatric Cardiology, National Taiwan University Hospital, Taipei, Taiwan Mei-Hwan Wu, M.D., Ph.D., FACC, FESC Professor of Pediatrics, National Taiwan University Hospital, President, Taiwan Society of Pediatric Cardiology, Taipei, Taiwan Yih-Sharng Chen, M.D. Associate Professor, Department of Cardiovascular Surgery, National Taiwan University Hospital, Taipei, Taiwan USA Satid Thammasitboon, M.D., M.H.P.E. Assistant Professor of Pediatrics, Associate Director, Pediatric residency program, West Virginia University School of Medicine, Department of Pediatrics, Pediatric Critical Care Section, Morgantown, WV, USA Invited Asia-Pacific Speakers Thivakorn Kasemsri, M.D., F.A.A.P Director, PICU, Covenant Children’s Hospital, Lubbock, Texas, USA Vietnam Nguyen Lan Hieu, M.D. Vietnam Heart Institute, Bach Mai Hospital, Hanoi, Vietnam. Nguyen Thanh Cong, M.D. Badinh, Hanoi, Vietnam. Thailand Boonchob Pongpanich, M.D. Professor Emeritus of Pediatrics, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Apichai Khongphatthanayothin, M.D. Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand Kritvikrom Durongpisitkul, M.D. Department of Pediatrics, Siriraj Hospital, Mahidol University, Bangkok, Thailand Manat Panamonta, M.D. Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand Pornthep Lertsapcharoen, M.D. Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand Suvipaporn Siripornpitak, M.D. Department of Radiology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Thanarat Layangool, M.D. Queen Sirikit National Institute of Child Health, Bangkok, Thailand Vachara Jamjureeruk, M.D. Bangkok Heart Hospital, Bangkok, Thailand Yuttapong Wongswadiwat, M.D. Pediatric cardiologist/intensivist, Queen Sirikit Heart Center of the Northeast, Khon Kaen University, Khon Kaen, Thailand General Information Congress Dates November 1-4, 2006 Congress Venue Bangkok Convention Center (BCC), Sofitel Central Plaza Hotel, Bangkok, Thailand Opening Ceremony & Welcome Cocktail Reception October 31, 2006, 18.00-22.00 hrs Official Language The official language of the congress is English. There will be no simultaneous translation. Exhibition An exhibition will be organized at the congress venue. There will be a display of pharmaceutical products to bring participants up to date with the most recent advancements in the field of Pediatric Cardiology and Cardiac Surgery. Non pharmaceutical products are also will be displayed. Accompanying Persons Accompanying person(s) must be registered (before August 15, 2006 US$ 100 and after August 15, 2006 US$ 120 per person) for admission to the Exhibits, Opening Ceremony, Complimentary Tour. Payment All prices quoted in this book are in US$. Payment in other currencies will not be accepted. By register online at www.pccs2006.com or download registration form to complete it as mail order and return to Congress Secretariat, the fees can be paid by Credit cards (VISA, Master and JCB Card). Please note that personal or company cheques are not accepted. Payment of fees must accompany all Registration form. The registration will be authorized only after receipt of the registration fee. There are credit card facilities for on site registration. Cancellation Policy Cancellation of your registration, Hotel reservation and Tour Arrangement should be made in writing to the conference organizer by September 1, 2006, in order to make the necessary amendments, stating the name and address of the bank and account name and number which you wish your refund to be send. Administration cost for each cancellation will be charged 30% of the total amount. Please note that the refund will be send after the congress. General Information Hotel Cancellation by Participants Participants may cancel their reservation by notifying the hotel 15 days before the scheduled arrival date. In the event that cancellation is not notified to the hotel as set forth herein, the hotel will charge the equivalent of charges for one night’s accommodation from their credit card or prepaid deposit. Certificate of Attendance A certificate of attendance will be available upon request during registration. Accreditation The scientific sessions have been accredited for continuing medical education (CME) 26.5 credits. Climate The climate is influenced by seasonal monsoon. There are three main seasons; rainy (May-October), winter (November-January) and summer (February-April). The average low temperature is approximately in low to mid 20 c and high temperature in mid 30 c. Transportations 1. BTS Sky Train stations can mostly be found in commercial areas of Sukhumvit, Silom, it is fast and safe. The sky train is divided into 2 lines: Sukhumvit line (On Nut StationMo Chit Station), and Silom lines (Saphan Taksin Station-National Stadium Station). The sky train has a minimum and maximum fare of 10-40 baht. Tourists may buy 30 day tourist tickets to save money, only 250 baht/10 trips, or day tickets, 100 baht (unlimited trips). The BTS Tourist Information Centers is open daily from 8.00 a.m. 8.00 p.m. at the following stations: Siam, Nana and Saphan Taksin. For more information,call: 0-2617-7340. 2. MRTA Subway is another comfortable and fast transportation choice. The beginning of “Chalerm Ratcha Mongkhon” line is Hua Lum Phong station (also the main train station in Bangkok), total of 18 stations, major stations at: The Queen Sirikit’s International Convention and Exhibition Center, Silom, Suan Lumpini, Asoke, Ratchada, Ladprao and Pahonyotin road. The Asoke and Silom stations are connected to the the BTS. There are also “Park & Ride” at Ladprao and Phra Ram 9 Stations. MRTA Subway has a minimum and maximum fare of 14-36 baht. The MRTA subway is open daily from 06.00 a.m. 12.00 midnight. For more information, call: 0-2624-5200. 3. Taxis in Bangkok are metered. They charge a minimum of 35 baht, for the first 3 kilometers, and approximately 5 baht per kilometer thereafter. When in Traffic the meter is timed and calculated in the fare. General Information 4. Tuk-Tuk, these three-wheeled ‘open-air’ motorised taxis are popular for short journeys. Fares must be bargained in advance. Minimum fares, for journeys of up to 3 kilometers, are approximately 30 baht. 5. Motorcycles Taxi, in rush hours, there is a lot of traffic jam, if your late and want to be on time you can try the motorcycle taxi. Fares must be bargained in advance. Minimum fares are 5 baht. You need to bargain the fare before taking the ride. Travels Cheques and Credit Cards Major credit cards, such as VISA and Master Card are widely accepted in hotels and shops. Traveller’s cheques can be conveniently cashed at all commercial banks and authorized money exchanges. Currency The Thai currency is baht which is divided into 100 satang. Coins are in 25 and 50 satangs, 1, 5, and 10 baht. Foreign currency may be exchanged at authorized exchanged banks, hotels and shops. Liability The Congress will not assume any responsibility for accidents, losses damages, delays or any modifications in the program caused by unforeseen circumstances. Non-Smoking Policy The Congress follows a non-smoking policy. No smoking every where during the congress, such as in the lecture or meeting rooms, exhibition area, poster presentation area etc. Emergency Clinic Located infront of elevator 4th Floor convention center. Registration Information Pre-registered delegates are required to produce their confirmation of registration at the registration counters to obtain their registration badges and congress kits. On-site registration will also be accepted at the registration counters and can be made in cash (baht or USD) Registration Hours Registration will be from 10.00 - 17.00 Tuesday, October 31, 2006, at the registration area, 4th Floor, Bangkok Convention Center (BCC), Sofitel Central Plaza Hotel, Bangkok, Thailand. Onsite Registration Fee Status Delegate Main-Congress Oneday US$450 US$150 Accompanying Person US$120 * Person registering as a student, postdoctoral and fellow must enclose a copy of their cards or a document from institutions. - Please use the registration form for registration. - Delegate, student, postdoctoral, fellow: Registration fee includes conference admission, proceeding and other materials, opening/closing ceremony/coffee or tea break. - Accompanying person: Registration fee includes opening/closing ceremony, reception and Royal Grand Palace Tour. Badges All delegates and accompanying persons are kindly requested to wear the name badges when attending any meeting or social gathering. Only delegates who are wearing the name badges will be admitted to the lecture halls. Please note: accompanying persons will not be admitted to the scientific session and exhibition. Name badges have been color-coded as follow: Pink Main congress Navy Blue One-day registration on November 1, 2006 Purple One-day registration on November 2, 2006 Orange One-day registration on November 3, 2006 Green Speaker, Moderator and Chairperson Yellow Committee Grey Accompanying persons Guidelines for Speaker’s Presentations General Information 1. Please proceed to the registration counter to collect your meeting materials. 2. The Slide Preview Room is provided for speakers to view your slides, disks and CDs before presentation. It is located on the 4th Floor adjacent fo the secretariat office. 3. You are requested to hand in your slides, disks and CDs at least one day before your scheduled presentation. Your co-operation in handling in your slides, disks and CDs early will ensure that the technicians will have sufficient time to load your presentations. 4. All speakers are requested to collect their slides, disks and CDs from the Slide Preview Room, preferable on the same day after presentation and not later than 5.00 pm the same day. The Organizing Committee will not be responsible for your slides, disks and CDs not collected within this period. Audiovisual Aids The rooms used for the presentations are equipped with - Dedicated Presentation PC Computer with floppy disk and CD-ROM equipped with Microsoft Office - Screen for single projection - A LCD projector - A laser pointer - 33 mm slide projector (as requested) Please note that - Universal fonts, such as Arial and Times New Roman, are preferable for the PowerPoint presentation. - Presentation on an Apple computer (Macintosh system) is not possible. Note: Presentation by slide 35 mm or any presentations, which do not use computer projection, will cause an inconvenient viewing for participants. PowerPoint presentation is the most recommended presentation tool. Instruction for Poster Presentation - The poster board is 120 cm in width x 240 cm in height. The suitable poster size is within 100 cm in width x 150 cm in height. - The abstract title, author names and institutions in large letters should be placed at the top of the poster followed by the headings: Background, Objectives, Materials & Methods, Results, and Conclusion to identify your poster layout. - The posters should be displayed in the poster areas throughout the period of the conference. Each poster board is marked with the number assigned to each abstract. Authors are requested to affix their posters at the number assigned to them during the official conference hours from 8.00 am on November 1, 2006 and 12.00 noon on November 4, 2006. - Pins, staplers, nails or any other sharp materials which might destroy the poster board are not allowed to use for affixing the poster. Two-side tape is the most recommended material for presentation. Please be noted that the posting material should be provided by the presenter. - Authors are requested to present at their posters during the poster sessions during coffee break. - Posters can be removed following the designated poster session if necessary. All posters not collected by November 4, 2006 12.00 noon will be disposed off. The conference assumes no responsibilities for the condition of posters not removed by the presenter. Saturday Nov4, 2006 Friday Nov3, 2006 Thursday Nov2, 2006 Wednesday Nov 1, 2006 Tuesday Oct31, 2006 C B Plenary 3: Cardiology from bench to bedside Plenary 2: Novel issues in cardiac surgery and noninvasive imaging Plenary 4: Towards the optimum care for pediatric and S36: Subpulmonary VSD congenital heart disease in AsiaPacific S37: Heterotaxy syndrome Abstract 8: FP15-FP21: Cardiac Surgery D Debate: Transcatheter valve therapy Abstract 7: FP43-FP49: Intervention C A S27: Hot topics in Cardiac CICU 2 B Abstract 4: FP29-FP35: General D S26: Sudden cardiac death in children Abstract 3: FP8-FP14: Cardiac Surgery C A S15: Rheumatic carditis revisited S4: Kawasaki disease D B S3: Nursing 1: care of child wiht CHD C S14: Pulmonary regurgitation in TOF S2: Surgical tips and techniques B Plenary 1: Children with heart disease in developing countries: How much can one do? 9:30-10:30 Young Investigator Award Presentation and Closing Ceremony Symposium S29: Nursing 2: Basic CHD S32: Nursing 3: Role of nursing in CHD 1 S31: SVT and Catheter ablation in CHD S30:Interventional club: “How to” S28: Cardiac service in developing countries Abstract 9: YIA1-YIA6 YIA Contest S21: Fetal Cardiology Revisited 1 Abstract 6: FP36-FP42: General & Arrhythmia Lunch Symposium3: Intervention symposium: VSD device closure S20: Grown Up CHD (GUCH) S19: Cardiac cath in Asia-Pacific 1 Abstract 5: FP50-FP56: General Cardiology S17: Surgery for TGA S18:Cardiac ICU in developing world S16: Heart Failure Lunch Symposium2: Eisenmenger Syndrome S10:Genetics in pediatric heart disease Abstract 2: FP22-FP28 Intensive Care S8: Cardiac Life Support (ECLS) S7:Advanced noninvasive imaging 14:00-15:30 S9: The Fontan Operation Lunch Symposium1: Valve repair in Children Registration 12:15-14:00 Abstract 1: FP1-FP7 Cardiac Surgery S6: Acquired heart disease in Asia-Pacific S5: Interventional tips and techniques 10:45-12:15 S35: Nursing 4: Role of nursing in CHD 2 S34: Device therapy in children S33: Intervention: Difficult cases S25: Fetal Cardiology Revisited 2 S24: Congenitally Corrected TGA S23: Cardiac cath in Asia-Pacific 2 S22: Hot topics for CICU I S13:Cardiomyopathy: What’s new? Hands-on: Workshop on Biodegradable Ring S12: CICU interactive case presentation S11: Advanced ECHO imaging 15:45-17:15 Scientific Program: “Towards the Optimal Care for Children with Heart Diseases in Asia-Pacific” A S1: Pulmonary Hypertension 08:00-9:30 A Room PS 1-22, PS115, PS121 PS 47-67, PS114, PS119, PS122 PS 92-120 Break PS 23-46 PS 68-91, PS116 Break Date Siam Niramit (Advance Booking) Committee dinner/Joe Louis Theater (Advance Booking) Opening ceremony & Reception 18:00-21:00 Program at a Glance Scientific Program Tuesday, October 31, 2006 10:30-17:00 18:00-19:00 19:00-21:00 Registration Opening ceremony Reception Wednesday, November 1, 2006 08:00-9:30 Symposium 1: PulmonaryHypertension Chairman: Dr. Edgardo E. Ortiz Co-Chairman: Dr. Alisa Limsuwan 1. Treatment of PH in CICU Dr. Dan Penny (Australia) 2. Treatment of Chronic PH Dr. James Yip (Singapore) 3. Testing of vasoreactivity in patient with PAH Dr. Geetha Kandavello (Malaysia) Room A Symposium 2: Meet the experts: Surgical tips and techniques Room B Chairman: Dr. Krishna Iyer Co-Chairman: Dr. Vichai Benjachalamas 1. Sano operation Dr. Shunji Sano (Japan) 2. Modified Norwood procedure Dr. Thomas L. Spray (USA) 3. RVOT reconstruction for first stage palliation of PA/VSD Dr. Jinfen Liu (China) Symposium 3: Nursing 1: The care of children with CHD Chairman: Rutja Phuphaibu, R.N., Ph.D. Co-Chairman: Ms. Bernadette McCormick (USA) 1. Postoperative care for children with heart disease Dr. Anthony Chang (USA) 2. Medications for pediatric patients after surgery for CHD Ms. Xiao-Rong, Gu (China) Ms. Lily Lihwa Hsu (China) 3. Sedation in children: What a nurse should know Dr. Neeti Chandra (India) Room C Scientific Program Symposium 4: Kawasaki disease Chairman: Dr. Hung Chi Lue Co-Chairman: Dr. Chule Thisyakorn 1. Epidemiology in non-Oriental: KD in Indonesia Dr. Najib Advani (Indonesia) 2. Vascular biology in Kawasaki disease Dr. Yoshihide Mitani(Japan) 3. Catheter and surgical treatment Dr. Teiji Akagi (Japan) Room D 09:30-10:30 Plenary1: Children with heart disease in developing countries: How much can one do? Room A Chairman: Dr. Shuji Sano Co-Chairman: Dr. Sukman Putra 1. Medical perspective Dr. Boonchob Pongpanich (Thailand) 2. Surgical Perspective Dr. Krishna Iyer (India) 10:30-10:45 Coffee break and Poster presentation 1: PS 1-22, PS115, PS121 (Adult CHD, Arrhythmia, ECHO, Pediatric CHD) 10:45-12:15 Symposium 5: Meet the expert: Interventional tips and technique Chairman: Dr. Yonghao Gui Co-Chairman: Dr. Khalilullah 1. Coil occlusion of AV fistula and collateral Dr. Teiji Akagi (Japan) 2. Stenting of coarctation of aorta Dr. Masood Sadiq (Pakistan) 3. Intervention for PDA closure Dr. Mohammad Omar Galal (Saudi Arabia) Room A Symposium 6: Acquired heart disease in Asia-Pacific 2006 Room B Chairman: Dr. Anita Khalil Co-Chairman: Dr. Chotima Pathamanand 1. Infective endocarditis in Pediatric Patients: Thailand Experience Dr. Vachara Jamjureeruk (Thailand) 2. Risk factor for coronary heart disease: management in childhood in developing countries Dr. Kalim Uddin Aziz (Pakistan) Scientific Program 3. Metabolic syndrome in children Dr. Masao Yoshinaga (Japan) Abstract 1: FP1-FP7 Cardiac Surgery Chairman: Dr. Katuhino Tatsuno Co-Chairman: Dr. Somboon Boonkasem Room C Abstract 2: FP22-FP28 IntensiveCare Chairman: Dr. Dan Penny Co-Chairman: Dr. Amit Varma Room D 12:15-14:00 Lunch Symposium1: Valve repair in Children Room A Chairman: Dr. Thomas L. Spray Co-Chairman: Dr. Pirapat Mokarapong 1. Value repair in congenital MS/MR Dr. Dong Man Seo (Korea) 2. Mitral and tricuspid valve repair using a Biodegradable Ring in pediatric population Dr. Afksendiyos Kalangos (Switzerland) 3. The Kalangos Ring in pediatric valve repair Dr. Mohamad Azhari Yakub (Malaysia) 14:00-15:30 Symposium 7: Advanced non-invasive imaging Chairman: Dr. Guo-ying Huang Co-Chairman: Dr. Rattanaporn Pornkul 1. The role of multistice CT in the diagnosis of CHD Dr. William C. L. Yip (Singapore) 2. Advance of cardiac MRI in the evaluation of CHD Dr. Suvipaporn Siripornpitak (Thailand) 3. 3D and 4D echo: Does it matter? Dr. David Sahn (USA) Room A Symposium 8: Cardiac Extracorporeal Life Support (ECLS) Chairman: Dr. Nguyen Thanh Cong Co-Chairman: Dr. Yuttapong Wongswadiwat 1. Ventricular Assist Devices (VAD) Dr. Anthony C. Chang (USA) 2. Post – operative ECLS Dr. Laurance L. Lequier (Canada) Room B Scientific Program 3. Organization of ECLS in Medical Center and its multi-application Dr. Yih-Sharng Chen (Taiwan) Symposium9: The Fontan Operation Chairman: Dr. Zhi-Wei Zu Co-Chairman: Dr. Samphant Ponvilawan 1. Lateral tunnel vs. extracardiac Fontan: Which is the best? Dr. Thomas L. Spray (USA) 2. Fontan in heterotaxy syndrome Dr. Hiromi Kurosawa (Japan) 3. Alternative treatments for unsuitable Fontan Dr. Roger B. Mee (Australia) Room C Symposium10: Genetics in pediatric heart disease Chairman: Dr. Yoshihide Mitani Co-Chairman: Dr. Pongsak Khowsathit 1. Marfan syndrome Dr. Heung Jae Lee (Korea) 2. Genetic basis of cardiac arrhythmias Dr. Chun-Wei Lu (Taiwan) 3. 22q11 deletion Dr. In-Sook Park (Korea) Room D 15:30-15:45 Coffee break and Poster presentation 2: PS 23-46 (Basic Science, Kawasaki disease) 15:45-17:15 Symposium 11: Advanced ECHO imaging Chairman: Dr. Sang Bum Lee Co-Chairman: Dr. Suthep Wanichkul 1. Novel ECHO parameter for cardiac functional evaluation Dr. David Sahn (USA) 2. Tele-Echo and its utility in Asia-Pacific: Experience in India Dr. Sunita Maheshwari (India) 3. Real-time 3D Echo for CHD Dr. Guo-ying Huang (China) Symposium 12: CICU interactive case presentation Chairman: Dr. Anthony C. Chang Co-Chairman: Dr. Pavathi Iyer 1. Case presentation from SCMC Dr. Zhuoming XU (China) Room A Room B Scientific Program 2. Case presentation from SCH Dr. Laurance L. Lequier (Canada) Hand-on session 1 (Surgery): Workshop on Biodegradable Ring Room C Chairman: Dr. Afksendiyos Kalangos Co-Chairman: Dr. Supreecha Tanamai Symposium13: Cardiomyopathy:What’s new? Chairman: Dr. Yonghao Gui Co-Chairman: Dr. Yupada Pongprot 1. Childhood cardiomyopathy: Experience in Australia Dr. James L. Wilkinson (Australia) 2. Cell therapy Dr. Christoff Stamm (Germany) 3. Heart transplantation Dr. Yih-Sharng Chen (Taiwan) Room D 18:00-21:00 Committee dinner/Joe Louis Theater (Advance Booking) Scientific Program Thursday, November 2, 2006 08:00-09:30 Symposium 14: Pulmonary regurgitation in TOF: Old Problems, New Answers Chairman: Dr. Pantpis Sakornpant Co-Chairman: Dr. William C. L. Yip 1. Surgical repair: How to avoid late complication Dr. Shunji Sano (Japan) 2. Physiologic determinant of PR Dr. Andrew N. Redington (Canada) 3. The role of the evaluation of PR/RV function post TOF repair Dr. Hyun Woo Goo (Korea) Room A Symposium 15: Rheumatic carditis revisited Room B Chairman: Dr. Luis Mabilangan Co-Chairman: Dr. Cherdchai Tontisirin 1. Evolution of ARF presentation: Does Jone’s criteria work well in high-prevalence countries ? Dr. Nigel Wilson (New Zealand) 2. The use of echocardiography in acute rheumatic fever. Dr. Manat Panamonta (Thailand) 3. RHD after 30 years of benzathine penicillin Dr. Hung Chi Lue (Taiwan) Abstract 3: FP8-FP14: Cardiac Surgery Chairman: Dr. Roger B.B. Mee Co-Chairman: Dr. Vichao Kojuranjit Room C Abstract 4: FP29-FP35: General Cardiology Chairman: Dr. Anna U. Rahayoe Co-Chairman: Dr. Masood Sadiq Room D 09:30-10:30 Plenary 2: Novel issues in cardiac surgery and non-invasive imaging Chairman: Dr. Chadsri Prachuabmoh Co-Chairman: Dr. Jinfen Liu 1. Tissue Engineering in CHD Dr. Hiromi Kurosawa (Japan) 2. Advanced ultrasound methods for procedural guidance in non-invasive cardiovascular imaging Dr. David Sahn (USA) Room A Scientific Program 10:30-10:45 Coffee break and Poster presentation 3: PS 47-67, PS114, PS119, PS122 (Rheumatic, Endocarditis, Cardiomyopathy, Genetics, Intensive Care) 10:45-12:15 Symposium 16: Heart Failure Chairman: Dr. Virojna Sueblinvong Co-Chairman: Dr. Anthony C. Chang 1. Basic mechanism and treatments of heart failure in children Dr. Makoto Nakazawa (Japan) 2. LCOS in CICU without mechanical support: What is possible? Dr. Pavathi Iyer (India) 3. Heart failure in GUCH Dr. Andrew N. Redington (Canada) Room A Symposium 17: Surgery for TGA Room B Chairman: Dr. Dong Man Seo Co-Chairman: Dr. Kriengchai Prasongsukarn 1. Aortic translocation in TGA/VSD/PS Dr. Zhi-Wei Xu (China) 2. TGA with abnormal coronary pattern: Technique and precaution Dr. Thomas L. Spray (USA) 3. Long-term FU post arterial switch: What to be concern Dr. James L. Wilkinson (Australia) Abstract 5: FP50-FP56: General Cardiology Chairman: Dr. Nigel Wilson Co-Chairman: Dr. Chalermkiet Tantrakool Room C Abstract 6: FP36-FP42: General Cardiology and Arrhythmia Chairman: Dr. Michael J. Silka Co-Chairman: Dr. Mei-Hwan Wu Room D 12:15-14:00 Lunch Symposium 2: Eisenmenger Syndrome Chairman: Dr. Kritvikrom Durongpisitkul Co-Chairman: Dr. Koichiro Niwa 1. Management of Eisenmenger Syndrome: Current practice Dr. Geetha Kandevello (Malaysia) 2. BREATH 5 Dr. Michael A Gatzoulis (UK) Room A 14:00-15:30 Symposium 18: Cardiac intensive care in developing world Chairman: Dr. Thivakorn Kasemsri Room A Scientific Program Co-Chairman: Dr. Lourance L. Leguier 1. Experience in India Dr. Pavathi Iyer (India) 2. Experience in China Dr. Zhuoming XU (China) 3. Experience in Thailand Dr. Yuttapong Wongswadiwat (Thailand) Symposium 19: Cardiac catheterization in Asia: Different point of view 1 Room B Chairman: Dr. Sukman Putra Co-Chairman: Dr. Seong-Ho Kim 1. A staged catheter-surgery approach in TOF/PA Dr. Seong-Ho Kim (Korea) 2. From bench to clinical practice: Lesson learned from platinum coated Notinol device Dr. Pornthep Lertsapcharoen (Thailand) 3. PDA stenting: what is the indication and outcome? Dr. Mazeni Alwi (Malaysia) Symposium 20: Grown Up Congenital Heart disease (GUCH) Room C Chairman: Dr. Heung Jae Lee Co-Chairman: Dr. Aungkana Gengsakul 1. Life-long concern in CHD: Experience of the GUCH clinic in Asia-Pacific Dr. James Yip (Singapore) 2. Exercise toterance in GUCH after the surgery Dr. Michael A. Gatzoulis (UK) 3. Pregnancy related issues in women with congenital heart disease Dr. Koichiro Niwa (Japan) Symposium 21: Fetal Cardiology Revisited 1 Room D Chairman: Dr. Samuel Menahem Co-Chairman: Dr. Jarupim Soongswang 1. Fetal cardiac pathology Dr. Andrew Cook (UK) 2. Impact of fetal echocardiography: Changing pattern of congenital heart disease Dr. In-Sook Park (Korea) 3. Progression of CHD during fetal life Dr. Yasuki Maeno (Japan) Scientific Program APPCS Business Meeting Chairman: Dr. Krishna Iyer Secretary: Dr. Apichai Khongphatthanayothin Kamphengphet 15:30-15:45 Coffee break and Poster presentation 4: PS 68-91, PS116 (Intervention, Nursing, Surgery) 15:45-17:15 Symposium 22: Hot topics for CICU I Room A Chairman: Dr. Ungkab Prakanrattana Co-Chairman: Dr. Sunita Maheshwari 1. Renal replacement therapy Dr. Laurance L. Lequier (Canada) 2. LCOS in CICU Dr. Zhuoming XU (China) 3. Expectation and outcome for Congenital Heart Surgery Programs Dr. Thivakorn Kasemsri (USA) Symposium 23: Cardiac catheterization in Asia: Different point view 2 Room B Chairman: Dr. Teiji Akagi Co-Chairman: Dr. Kritvikrom Durongpisitkul 1. PFM coil for VSD occlusion: A real alternative treatment modality Dr. Trong-Phi Le (German) 2. Difficult case for PDA closure Dr. Jou-Kou Wang (Taiwan) Symposium 24: Congenitally Corrected TGA Room C Chairman: Dr. James L. Wilkinson Co-Chairman: Dr. Anant Khositseth 1. Systemic RV: Natural History Dr. Michael A. Gatzoulis (UK) 2. The management of Congenitally Corrected Transposition of Great Arteries Dr. Roger B. B. Mee (Australia) Symposium 25: Fetal Cardiology Revisited 2 Room D Chairman: Dr. Samuel Menahem Co-Chairman: Dr. Vachara Jamjureeruk 1. Pre- and perinatal management of fetal arrhythmia Dr. Yasuki Maeno (Japan) 2. The management implication following the diagnosis of fetal heart anomalies Dr. Samuel Menahem (Australia) Scientific Program APPCS Business Meeting Chairman: Dr. Krishna Iyer Secretary: Dr. Apichai Khongphatthanayothin 18:00-22:00 Siam Niramit (Advance Booking) Kamphengphet Scientific Program Friday, November 3, 2006 08:00-09:30 Symposium 26: Sudden cardiac death in children Room A Chairman: Dr. Eun-Jung Bae Co-Chairman: Dr. Somkiat Sopontammarak 1. Risks and mechanisms of sudden death following surgical treatment of congenital heart disease Dr. Michael J. Silka (USA) 2. The long QT syndrome Dr. Masao Yoshinaga(Japan) 3. Sudden death in patients with right atrial isomerism Dr. Mei Hwan Wu (Taiwan) Symposium 27: Hot topics in Cardiac CICU 2 Room B Chairman: Dr. Oranuch Kyokong Co-Chairman: Dr. Neeti Chandra 1. Haemodynamic monitoring: tool or toy? Dr. Dan Penny (Australia) 2. Cardiopulmonary interaction: How principles become practices? Dr. Satid Thammasitboon (USA) 3. Nutritional support and immunocompetence Dr. Amit Varma (India) Abstract 7: FP43-FP49: Interventional Cardiology Chairman: Dr. Geoffrey Lane Co-Chairman: Dr. Jou-Kou Wang Room C Abstract 8: FP15-FP21: Cardiac Surgery Chairman: Dr. Shunji Sano Co-Chairman: Dr. Chareonkiat Roekkliang Room D 09:30-10:30 Plenary3: Cardiology from bench to bedside Room A Chairman: Dr. Makoto Nakazawa Co-Chairman: Dr. In-Sook Park 1. Pathologist’s bench: A New Look at Cardiac Development Dr. Andrew Cook (UK) 2. Cardiologist’s aspect of genetics of congenital CV disease: From molecular bench to bedside Dr. Makoto Nakazawa (Japan) Scientific Program 10:30-10:45 Coffee break and Poster presentation 5: PS 92-120 (Surgery) 10:45-12:15 Symposium 28: Children cardiac service in developing countries: Asia-Pacific experience Room A Chairman: Dr. Boonchob Pongpanich Co-Chairman: Dr. Sukman Putra 1. Pakistan Dr. Kalim Uddin Aziz (Pakistan) 2. Indonesia Anna U. Rahayoe (Indonesia) 3. The “Heart to Heart” project Dr. Heung Jae Lee (Korea) Dr. Thanh Cong Nguyen (Vietnam) Abstract 9: YIA1-YIA6: Young Investigator Award Contest Chairman: Dr. Edgardo E. Ortiz Co-Chairman: Dr. Chaisit Sangtawesin Room B Symposium 29: Nursing 2: Basic CHD Chairman: Rutja Phuphaibu, R.N., Ph.D. Co-Chairman: Ms. Lily Lihwa Hsu 1. Acyanotic CHD Ms. Li-Juan Fu (China) Ms. Lily Lihwa Hsu (China) 2. Cyanotic CHD Ms. Bernadette McCormick (USA) Room C 12:15-14:00 Lunch Symposium 3: Intervention symposium: VSD device closure Room A Chairman: Dr. Mazeni Alwi Co-Chairman: Dr. Mohammad Omar Galal 1. Introduction Dr. Kritvikrom Durongpisitkul (Thailand) 2. What does the Interventionist need to know about the anatomy of VSD? Dr. James L. Wilkinson (Australia) 3. Transcatheter closure of VSD: Results and Complications Dr. Ziyad M. Hijazi (USA) 14:00-15:30 Symposium 30: Interventional club: “How to” Chairman: Dr. Mosood Sadiq Room A Scientific Program Co-Chairman: Dr. Trong-Phi Le 1. Transcatheter closure of ASD “tips and techniques for large ASD” Dr. Geoffrey Lane (Australia) 2. Transcatheter closure of VSD: Asian perspective Dr. Mazeni Alwi (Malaysia) 3. Technique of balloon dilation of aortic coarctation Dr. Mohammed Omar Galal (Saudi Arabia) Symposium 31: SVT and Catheter ablation in CHD Room B Chairman: Dr. Mei Hwan Wu Co-Chairman: Dr. Preecha Laohakunakorn 1. Conduction system in CHD Dr. Andrew Cook (UK) 2. SVT in complex 2-ventricle CHD: Ebstein, L-TGA, D-TGA post Mustard Dr. Michael J. Silka (USA) 3. SVT in complex single ventricle and post Fontan operation Dr. Eun-Jung Bae (Korea) Symposium 32: Nursing 3: Role of nursing in the care of CHD 1 Room C Chairman: Ms. Bernadette McCormick (USA) Co-Chairman: Rutja Phuphaibul, R.N., Ph.D. 1. Patient and family education Ms. Bernadette McCormick (USA) 2. Support for family with in a child with CHD Ms. Lily Lihwa Hsu (China) 15:30-15:45 Coffee break 15:45-17:15 Symposium 33: Interventional symposium: Difficult cases presentation Chairman: Dr. Ziyad M. Hijazi Co-Chairman: Dr. Geoffrey Lane 1. Transcatheter Closure of PDA: Some problems & difficulties Dr. Sukman Putra (Indonesia) 2. Migrated stent and repositioning Dr. Seong-Ho Kim (Korea) 3. VSD and AV block Dr. Kritvikrom Durongpisitkul (Thailand) 4. Coil occlusion of VSD Dr. Nyuyen Lan Hieu (Vietnam) Room A Scientific Program Symposium 34: Device therapy in children Room B Chairman: Dr. Apichai Khongphatthanayothin Co-Chairman: Dr. Eun Jung Bae 1. Controversial indications for pacemaker implantation in children Dr. Michael J. Silka (USA) 2. CRT in children Dr. Mei Hwan Wu (Taiwan) 3. Pediatric AICD: A review Dr. Apichai Khongphatthanayothin (Thailand) Symposium 35: Nursing 4: Role of nursing in the care of CHD 2 Room C Chairman: Ms. Lily Lihwa Hsu Co-Chairman: Rutja Phuphaibul, R.N., Ph.D. 1. Long-term outcomes in patients with congenital heart defects Ms. Bernadette McCormick (USA) Scientific Program Saturday, November 4, 2006 08:00-9:00 Debate 1: Transcatheter valve therapy (weird or wonderful?) Chairman: Dr. Mohammad Omar Galal Co-Chairman: Dr. Geoffrey Lane 1. Pro Dr. Ziyad M. Hijazi (USA) 2. Con Dr. Andrew N. Redington (Canada) 3. Rebuttal Room A Symposium 36: Subpulmonary VSD, when to repair? Chairman: Dr. Apichart Nana Co-Chairman: Dr. Nguyen Lan Hieu 1. Natural history and echocardiographic evaluation Dr. Thanarat Layangool (Thailand) 2. Subpulmonary VSD: When and how to repair the AR Dr. Katuhino Tatsuno (Japan) Room B Symposium 37: Heterotaxy syndrome Chairman: Dr. Pongpira Suwannakul Co-Chairman: Dr. Najib Adani 1. The essence of isomerism of the atrial appendages, and its relationship to visceral heterotaxy Dr. Andrew Cook (UK) 2. Arrhythmia in heterotaxy syndrome Dr. Eun Jung Bae (Korea) Room C 09:00-10:30 Plenary 4: Towards the optimum care for pediatric and congenital heart disease in Asia-Pacific Room A Chairman: Dr. Krishna Iyer Co-Chairman: Dr. Boonchob Pongpanich 1. Towards the optimum care of pediatric and congenital heart disease in the next decade Dr. Roger B. B. Mee (Australia) 2. The future of cardiac catheterization Dr. Ziyad M. Hijazi (USA) 3. Delivery of care in congenital heart programs: past, present and future Dr. Andrew N. Redington (Canada) Scientific Program 10:30-11:00 Young Investigator Award Presentation and Closing Ceremony Room A Chairman: Dr. Boonchob Pongpanich Co-Chairman: Dr. Chaisit Sangtawesin YIA Finalist Presentation and President’s closing remarks Dr. Krishna Iyer, President Host country closing remark and presentation of the next host country Dr. Boonchob Pongpanich, host country Dr. Lee Sang Bum, next host country (Korea) 11:00-11:15 Coffee break Remark: Topics & Speakers are subjected to change according to availability. The First Asia-Pacific Congress of Pediatric Cardiology and Cardiac Surgery November 1-4, 2006 SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S1(1/3) PULMONARY HYPERTENSION ON THE CARDIAC INTENSIVE CARE UNIT. Dan Penny, M.D., Ph.D, FRACP Director of Cardiology, The Royal Children’s Hospital, Melbourne, Australia A number of congenital heart lesions, particularly those resulting in a left-to-right shunt or pulmonary venous hypertension are associated with pulmonary arterial hypertension. Pulmonary vascular resistance is commonly increased during cardiopulmonary bypass and in susceptible patients, the post-bypass period may be complicated by pulmonary hypertension and in some, pulmonary hypertensive crises. It is now well-recognised that pulmonary hypertension after cardiac surgery is an important risk factor for morbidity and mortality and is an important determinant of ICU requirement. However, severe post-operative pulmonary hypertension is now much less common than before, because of timely surgery for lesions with left-to-right shunt and recent improvements in cardiopulmonary bypass, including the use of modified ultrafiltration. Severe episodic pulmonary hypertension after cardiac surgery is characterised by increases in central venous and pulmonary arterial pressures, reductions in left atrial pressure and oxygen saturation and ultimately reductions in systemic arterial pressure … the so-called ‘pulmonary hypertensive crisis’. These haemodynamic changes are commonly associated with changes in lung mechanics with acute increases in lung stiffness, a sign commonly mistaken for the development of obstruction of the endotracheal tube. Good basic intensive care, involving adequate analgesia, and in particular optimised ventilator strategies are the mainstay of prevention and treatment. However, the use of newer agents, based on increased understanding of the role of the endothelium in regulating the function of vascular smooth muscle has transformed our care. Nitric oxide (NO) inhalation has been one of the most important advances in cardiac intensive care over the last decade or so. NO is a selective and potent pulmonary vasodilator which when used appropriately can be life-saving. It is now recognized that even low inhalation rates (of around 2-5 ppm) can have a profound pulmonary vasodilator effects. However a number of problems with its use remain. First, it is unclear as to when exactly NO should be commenced and units differ considerably in their threshold indications. Second, some patients do not respond well to its application and third, rebound pulmonary hypertension after its withdrawal remains an important problem. The threshold indications for the initiation of NO inhalation remain ill-defined and different units have markedly different protocols for its commencement. While some papers in the literature point towards a clinical benefit for using NO to prevent pulmonary hypertension in at-risk patients, some protocols recommend its use only where pulmonary hypertension is associated with significant alterations in systemic haemodynamics. PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 In patients who do not respond to inhaled NO an important consideration is whether there is an anatomic reason for the elevated pulmonary arterial pressure. Having excluded a large vessel mechanical problem, for the lack of response, other agents may be considered, including the phosphodiesterase inhibitor sildenafil or possibly an endothelin agonist (although both approaches remain experimental). Rebound pulmonary hypertension can by an important problem. It may occur, even in patients where NO inhalation did not result in therapeutic benefit, in the first place. Rebound appears to be related to downregulation of NO synthase activity, possibly related to superoxide formation. In most patients, rebound may be prevented by cautious withdrawal of NO to concentrations of less than 1ppm before stopping. Recently, we have performed a randomized trial which demonstrated that a single dose of oral sildenafil before stopping inhaled NO may prevent rebound. Other approaches which are of more experimental use are antioxidants and endothelin antagonists. In conclusion, the problem of pulmonary hypertension is now less common than before in patients on the cardiac intensive care unit. However, when it occurs, it carries with it, significant morbidity and mortality. Timely surgery, meticulous attention during cardiopulmonary bypass and good, basic intensive care, are the mainstay of prevention and treatment. Where these measures are insufficient, inhalation of NO may be indicated. Patients with elevated right ventricular or pulmonary arterial pressure who do not respond to NO treatment should be carefully assessed for residual haemodynamic lesions. SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S1(2/3) Treatment of Chronic PH James Yip, M.D. The Heart Institute, National University Hospital, Singapore PCCS 2006 Testing of vasoreactivity in patient with PAH Geetha Kandavello, M.D. Consultant Pediatric Cardiologist, Dept of Pediatric Cardiology Institute Jantung Negara, National Heart Institute, Kuala Lumpur, Malaysia SPEAKER’S ABSTRACTS November 1, 2006 S1(3/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S2(1/3) Sano operation Shunji Sano, M.D., Ph.D. Professor and Chairman Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 S2(2/3) Modified Norwood procedure Thomas L. Spray, M.D. Children’s Hospital of Philadelphia, Philadelphia, PA, USA. SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S2(3/3) RVOT reconstruction for first stage palliation of PA/VSD Jinfeng Liu, M.D. Chief Physician, Shanghai Children Medical Center, Shanghai, China Background:To evaluate restoration of anterior pulmonary circulation with Gore-tex conduit in first-stage palliation of pulmonary atresia with ventricular septal defect. Methods: Between January 2003 and July 2005,14 patients underwent operation?male 8 cases?female 6 cases.Mean age was 32 30m(4-53m),weight 14 6kg(3-14.5kg), SpO269 16%(58-88%) preoperatively. PA/VSD is classified into four types according to the development of main pulmonary artery(MPA) and the presence of major aortopulmonary collateral vessels(MAPCVs).Type I,duct dependent and MPA present.Type II, duct dependent and MPA not present. Type III,dual supply by true pulmonary artery and MAPCVs. Type IV?no true pulmonary artery,only MAPCAs. Left and right pulmonary artery should be dissected freely. Ductus arterious was ligated immediately after commencing bypass. Left or right pulmonary arery less than 2 to 3mm was enlarged to 4 to 5mm in 6 cases. There was no confluence between left and right pulmonary artery whose posterior wall was end-end anastomosed and anterior wall was enlarged by pericardial in 4 cases .Right pulmonary anterior wall was incised and reversed as posterior wall cause there was a long distance between left and right pulmonary artery in 1 case. Then new anterior wall was enlarged by pericardial. The distal Gore-Tex conduit was fashioned to the true pulmonary artery. A small incision less than 1 to 2mm of conduit diameter is made in the infundibulum of right ventricle. The proximal Gore-Tex conduit was sutured to it.For 2 cases of less than or equal 7kg 6mm conduit is used, 4 cases of less than 9kg 8mm used,6 cases less than 14kg 10mm used,2 cases of large than or equal 14kg 13mm used. Modified ultralfiltration was performed after CPB. Results:Type I 5 patients,type II 4 patients ,type III 5patients.9 patients performed with parallel cardiopulmonary bypass,mean time was 61 20min(34-96min) 5 patients performed with heart arrest, mean aortic clamp time was 46 24min(18-80min),mean cardiopulmonary bypass time 95 33min(47-123min) Mean ventilator time was 31 17h(19-74h),ICU stay time 4 2d(2-8d) HR and AoSP were lower 24h and 48h than 6h postoperatively(P<0.05). After 6h,24h and 48h of postoperation, HR was 148 20/min,139 18/min and 143 8/min relatively. Aortic systolic pressure 97 13mmHg, 94 22mmHg and 91 12mmHg.Aortic diastolic pressure 57 8mmHg,53 10mmHg and56 8mmHg.The HR and aortic systolic pressure after 24h and 48h of postoperation were significantly less than 6h(P<0.05). After 6h,24h and 48h of postoperation,PaO2 was 57 13mmHg,59 12 mmHg and 58 7 mmHg PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 relatively.PaCO2 38 4 mmHg,35 4 mmHg and 34 7 mmHg .,SaO2 0.88 0.07,0.9 0.05 and 0.86 0.04.There were no significant difference in PaO2 and SaO2 6h,24h and 48h postoperatively. 1 type I case complicated with right pleural effusion after removal of ventilator and was drained, recovered 3 days later.1 type I case complicated with pericardial effusion after removal of ventilator and decreased obviously 4 days later. 1 type III case was reoperated because of bleeding after returning ICU. Fellow ECHO showed VSD double shunt ,mean gradient of right venticle-pulmonary artert was 42 17mmHg(10-68mmHg). EKG showed sinus rhythm.Heart function I 3 cases, II 8 cases, III cases 3 cases. Conclusion: Restoration of anterior pulmonary circulation with Gore-tex conduit can maintain pulmonary blood flowin rightly, stabilize hemodynamics, remove ventilator favorably providing good condition for two-stage procedure. SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S3(1/3) Postoperative care for children with heart disease Anthony C. Chang, M.D., MBA CHOC Heart Institute Children’s Hospital of Orange County, CA, USA PCCS 2006 Medications for Pediatric Patients after Cardiovascular Surgery for Congenital Heart Diseases Xiao Rong Gu1, RN., BSN Lily Hsu2, RN., MSN Ping Ping He1, RN., BSN Shanghai Children’s Medical Center Affiliated with Shanghai Jiao Tong University School of Medicine1 Shanghai Jiao Tong University School of Nursing, Project HOPE2 Pediatric patients with Congenital Heart Disease (CHD) post surgery require various medications to maintain their cardiac function and to promote the stability of their physiological parameters. Children who received cardiovascular surgery frequently suffered from pain, anxiety, cardiac arrhythmias, low cardiac output, high pulmonary arterial hypertension, low systematic pressure, low urine output, heart block, or heart failure, etc.. These condition required several medications in order to maintain the cardiac output, to lower the pulmonary hypertension, and to promote their comfort post surgery in Cardiac Intensive Care Unit. While patients receive several important medications, it is essential for nurses to realize the drug action, side effects, rational to use, and pharmacokinetics property of these medications in order to provide prompt care and to avoid complication associated with these medications. Nursing assessment becomes essential and the effect of medication needs to be timely documented. It is essential for nurses to recognize patients’ clinical status in critical care setting. The nurse's ability to understand children’s clinical condition and medications is enhanced by an understanding of the principles of cardiac anatomy and physiology, as well as developmental changes in cardiac function (Miller-Hoover, 2003). Beta-blockers, angiotensin-converting enzyme inhibitors, calcium channel blockers are commonly used during postoperative period. Frequently used medications in CICU are Lidocaine, Amiodarone, Amrinone, Adenosine, Capropril, Digoxin, Dopamine, Dobutamine, Furosemide (Lasix), Nipride, Prostaglandin, and Nitroglycerin. Each medication’s side effects and nursing intervention need to be monitored and addressed carefully. Corticosteroids are often used in CICU during postoperative period with aim to reduce the postoperative inflammatory response. Inotropic support is provided to ensure the stability of homodynamic status. Phosphodiesterase inhibitors, such as milrinone, are used after the Norwood procedure because of their combined inotropic and vasodilator properties (Soetenga & Mussatto, 2004). Dobutamine is used to support myocardial contractility and Dopamine is commonly used in CICU for blood pressure support, and promoting the cerebral and renal perfusion. Sodium nitroprusside is for decreasing afterload; SPEAKER’S ABSTRACTS November 1, 2006 S3(2/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 nitroglycerin is to improve left ventricular function by lowering preload from vasodilatation. ACE inhibitor is used for afterload reduction. Also, digoxin is frequently used to support the function of the single ventricle, a diuretic to prevent fluid retention is also used for children at home, an Captopril will be ordered as an after-load-reducing agent to decrease the workload of the single ventricle, and aspirin or enoxaparin sodium (Lovenox) to decrease the risk of thrombotic complications (Soetenga & Mussatto, 2004). For patients’ agitation and post-operative pain, Fentany l5-10 micro/g/kg per hour with neuromuscular blocker benzodiazepines (lorazepam) are used frequently in CICU. Children in the post-operative stages need to receive a comprehensive discharge teaching. Medication is one of key topic to be covered by nurse at bedside. Prompt nursing intervention is important for taking care of children who receive various cardiac medicines during post-operative stage. These interventions include assess the level of consciousness, monitor serum electrolytes, BUN, serum creatinine, live enzyme, monitor intake & output, and assess any significant bodyweight changes. In addition, nurses need to assess parameters of cardiovascular functioning on a regular basis, any change of cardiac rhythm on EKG need to report immediately. The fluctuation of Blood pressure and weak pulse rate may indicate patient’s condition in instable, heart murmur my indicate regurgitation post surgery, course lung sounds will direct to more efficient respiratory management, weak peripheral pulses need to monitor homodynamic status, presence of edema (especially in dependent areas) and level of jugular venous distention all need to be carefully assessed for the possibility of heart failure. Patients’ medication adjustment should be based on patient clinical status, postoperative cardiac function, and should use a micro drip intravenous set-up to delivery the medication. Nurses need to label all intravenous infusions carefully, and keep accurate records of drug additives to prevent inadvertent medication errors. All medication calculation needs to be double checked, especially medications that may affect systematic pressure and cardiac output. Nurses also need to teach patients and families about the desired effects and common side effects of prescribed drugs. For discharge teaching, nurses need to teach parents to keep all medications out of reach of children, keep all health care providers informed of all medications being used, emphasize to patients the importance of taking drugs as ordered, not discontinuing drugs without consulting with the physician, not sharing drugs with friends of relatives, and not self-medicating with over-the-counter drugs unless first approved by the physician. Teach patients about the signs of drug allergies. If patients take corticosteroids or anticoagulants, instruct children to wear medical identifications. Instruct parents to let children take drugs with the food to lessen the gastric irritation and this may improve the compliance of the home medications. In addition, using mealtimes as a reminder is often a way to help patients remember prescribed drugs. Children post cardiac surgery will experience various cardiac medications. It is nurse’s responsibility to get familiar with the medication. Continue education and reading key article are ways to enhance the knowledge on medications that are used in CICU. Carefully assess patients’ status during pre-operative and post-operative and document adequate medications record are essential for understanding the status of the patients. PCCS 2006 Sedation in children: What a nurse should know Neeti Chandra, M.D. New Delhi, India SPEAKER’S ABSTRACTS November 1, 2006 S3(3/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S4(1/3) Epidemiology in non-Oriental: KD in Indonesia Najib Advani, M.D., MMed Consultant Pediatric Cardiologist University of Indonesia, Jakarta, Indonesia Kawasaki disease is still not very well known in Indonesia, a country with 220 million populations. The estimated annual incidence is approximately 5000 cases, but the number of diagnosed cases is less than 100 per year. The first case series consisted of 27 Kawasaki patients in Indonesia was reported at the Eighth International Kawasaki Disease Symposium in SanDiego, 2005. In order to introduce the disease to the community and medical personnel, we give lectures to undergraduate medical students, case presentations to the residents, talks in radio stations and TV channels, publish book and flyers, also write articles in public magazines and newspapers on Kawasaki disease. As a result, the number of diagnosed cases is increasing gradually. However it is still far from expected. We are worried of the undiagnosed cases. A lot of effort is needed in order to make all the medical practitioners aware of the disease. PCCS 2006 Vascular biology in Kawasaki disease: Lessons learned from long-term issues. Yoshihide Mitani, M.D. Mie University School of Medicine, Japan Background: Coronary sequelae which persist after Kawasaki disease (KD) have been associated with obstructive changes of the lesions and coronary vascular events in adolescents and young adults. However, little is known about the association between sequelae late after KD and inflammatory markers, which are potential mediators and markers for atherogenesis. Methods and Results: Cross-sectional study was performed to test the hypothesis that coronary sequelae are associated with elevated levels of inflammatory markers in patients late after KD?(mean time interval after the onset:10y10m). Levels of high sensitivity Creactive protein (CRP), serum amyloid-A (SAA), interleukin-6, and soluble intercellular adhesion molecule-1 were measured in 4 groups (n=80): referent (n=15) and KD subgroups with normal coronary arteries from the onset (n=27), with regressed aneurysms (n=18), and with coronary artery lesions, such as persistent aneurysms, stenosis, and occulusion (n=20). CRP levels were significantly elevated in a KD subgroup with coronary artery lesions, compared with the referent or other KD subgroups, as analyzed by analysis of variance, and analysis of covariance after adjustment for a confounding factor body mass index. Levels of CRP, SAA and interleukin-6 were positively correlated. Stepwise regression and logistic regression analyses support the association between the persistence of coronary artery lesions and the levels of CRP and SAA. Conclusions: Results demonstrate that the persistence of coronary lesions late after KD was independently associated with levels of CRP and SAA, suggesting inflammation may be a novel functional aspect of coronary artery diseases late after KD. SPEAKER’S ABSTRACTS November 1, 2006 S4(2/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S4(3/3) Catheter and surgical treatment of KD Teiji Akagi, M.D. Associate Professor Cardiac Care Unit, Okayama University Hospital Okayama, Japan Even if the perfect IVIG treatment was undertaken in the acute stage of illness, we could not reach the complete avoidance of coronary artery complications. The most striking feature of coronary artery abnormality is the change of size or shape of aneurysm. About 50% of coronary aneurysms regress within 2 years. On the other hand, coronary artery stenosis occurred in 4% of all patients, or in 20% with coronary aneurysms in the followup period. Myocardial infarction occurred in about 1% of patients, one-third of those patients died due to cardiac shock. It is important to know that half of the fatal cases did not demonstrate any ischemic findings before the onset of myocardial infarction. In this regard, the result of coronary bypass grafting in children with Kawasaki disease is not reached sufficient level. More than 90% of 15 years graft patency rate was expecting if intrathoracic arterial bypass surgery underwent older than 12 years old, however, 15 years graft patency rate decreased 65% in children under 12 years old even if the arterial graft was selected. In fact, the most of candidates of coronary bypass surgery are children younger than 12 years old. Especially in these young children, 15 years graft patency is not enough for their long future life. During past 10 years, the clinical experience of catheter interventional treatment in Kawasaki disease has been gradually increasing. These are including balloon angioplasty, stent implantation, rotational ablation, and transluminal coronary revascularization. Because the coronary artery stenosis in Kawasaki disease commonly involves severe calcification in contrast with adult atherosclerotic coronary artery lesions, the indication or technique of catheter intervention for adult patients cannot be directly employed in Kawasaki disease patients. Satisfactory acute results for coronary balloon angioplasty were obtained in patients with a relatively short interval from the onset of disease, especially within 6 years, however the incidence of restenosis after angioplasty was still high. Rotational ablation may be the most appropriate catheter intervention for Kawasaki disease. The advantage of rotational ablation is the high success rate, even in patients with calcified coronary artery stenosis. Stent implantation requires larger arterial access and is not possible in younger children. Care should be paid to the detection of new aneurysm formation, as the formation of new aneurysms was associated with the use of additional balloon angioplasty using high pressure balloon inflations. Anticoagulation or anti-platelet regimens are essential for the longterm management. Above procedures should be performed at selected institutions with experience in coronary interventional procedures by highly experienced interventional cardiologists. Cooperation PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 between pediatric and adult cardiologists is essential for the optimal decision making in this setting. Patients should be referred by a cardiologist, who has fully knowledge of not only the catheter intervention but also the clinical feature and the natural course of this disease. The indications for catheter intervention and appropriate anti-thrombus regimen after the interventional catheterization require further clarification in the setting of Kawasaki disease, based on the natural history of coronary artery lesion especially vascular endothelial function. SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 P1(1/2) Problems of Children with Heart Disease in Developing Countries, How Much Can One Do? Boonchob Pongpanich M.D.,FACC. President of the Cardiac Children Foundation of Thailand The problems in developing countries (GNP per capita less than 10,000 US dollars) are inadequate medical personels, both specialists and general practitioners, causing a long waiting list and delay diagnosis and treatment. In addition the government health care budget are limited. Heart disease in infant and children are considered to be a low priority, because there are too many urgent health problems. With these limitation, pediatric cardiologist and cardiac surgeon has to work hard, usually with low income because most children with heart disease come from poor family, many has social problems. The most common type of heart disease in children is congenital heart disease (CHD) account for over 80 percents of cases and occur in almost one percent of live born infants, followed by rheumatic heart disease and Kawasaki disease. We also for seen future cardiovascular problems related to childhood obesity which are predispose to early type two diabetes mellitus and hypertension at a later age. This paper will focus mainly on CHD. It is estimated that each year there will be over 675,000 infants born with CHD in Asia-Pacific and most of them, over 600,000 are in the developing countries. Since CHD mostly unpreventable, approximately 20 % of CHD may die in infants if there is no treatment, many die before the diagnosis was made. To cope with these problems, training non-MD such as community nurses and public health personals of how to diagnose and early referral is very helpful. Mobile or extended pediatric cardiac clinic to the rural and remote area where there are no pediatric cardiologist is also very important. This could be done through a co-operation between public and private organization. In addition we should be cost conscious, both direct medical cost such as cost of investigation and treatment and social cost such as traveling, lodging and loss of work for parents. We should avoid too many appointments by co-operation with local MD. Thailand population is 63 millions, in 2003 there were 742,183 live born infants with over 7400 infants with CHD. Half of this number need intervention or surgery. There are 60 pediatric cardiologists and 20 cardiac surgeons who can perform pediatric cardiac surgery, very few can do surgery in the newborn and complex CHD. Each year less than 2500 infants and children could be able get surgical treatment. In the past few years with the support of the Cardiac Children Foundation and The Foundation for Pediatric Cardiac Surgery, several thousands children (over 5000) were operated during weekend or evening. These procedures could be done with the support of government budget through the PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 universal health care coverage which was started in 2002 and public donation. The Cardiac Children Foundation of Thailand has been organizing extended cardiac clinic for 13 consecutive years, with 38 trips, in 34 provinces. We have examined 3327 children,2262 with heart diseases and 1276 patients were sent for surgery or intervention. Conclusion. In developing countries with limit health man power and budget, we should try our best to improve coverage and quality care at a reasonable cost through a cooperation between public and private sectors. A co-operation among Asia-Pacific in training, service and research is necessary for future development. SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 P1(2/2) Children with heart disease in developing countries - How much can one do? ‘Surgical Perspective’ Krishna Iyer, M.Ch. Director, Pediatric & Congenital Heart Surgery, Escorts Heart Institute & Research Center, New Delhi, INDIA Congenital Heart Disease (CHD) continues to be a major pediatric health issue in developing countries. Surgical intervention remains the mainstay of treatment in CHD despite rapid advances in catheter-based interventions. Over the years appropriate and timely surgical intervention has dramatically altered the natural history of CHD, however surgery still comes at a significant cost. Socio-Economic considerations remain the major factor hampering the growth of this specialty in developing countries. There is a significant and sadly increasing disparity between health care facilities in the developed and the developing world. Since the prevalence of CHD is dependant on birth rates, developed countries with lower birthrates face a lower prevalence. At the same time they also have more resources to expend on their care. Health care expenditure by governments as a proportion of GDP is abysmal in developing countries and very little if any of this is available for high end health care like pediatric cardiac surgery. Increasingly, therefore in many developing countries pediatric cardiac care is shifting to the nongovernmental sector. Pediatric cardiac surgery in developing countries is particularly challenging because non-cardiac issues play a major role in determining surgical outcomes. High quality intensive care is difficult to establish and sustain leading to closure of many start-up surgical programs. Nevertheless, despite all these hurdles a lot can still be done. We need to take advantage of the large intellectual pool and relatively inexpensive labor force available in developing countries. Over 70% of congenital heart disease is treatable with relatively straightforward one time surgery.. The focus should therefore be on developing high volume centers managed by a few well-trained and skilled professionals making appropriate use of an easily available semi-skilled workforce. Cost cutting measures need to be aggressively pursued and these include use of locally made consumables and equipment, bulk purchase of imported items, careful recycling of selected items, rigorous screening of patients preoperatively and careful planning of surgical procedures to reduce post operative complications. Funding is a constant problem and every available source of funding needs to be tapped – government, charity organizations, world health bodies, philanthropists, religious bodies, corporate bodies, rich individuals and other revenue-generating programs within the same hospital. Dedicated and goal-directed team-work is the key to success in any pediatric cardiac program and if cardiac health care professionals in developing countries join hands and work towards a common goal a lot can be achieved despite the daunting drawbacks. PCCS 2006 Teiji Akagi, M.D. Cardiac Care Unit Okayama University Hospital, Japan Coronary arteriovenous fistula (CAVF) is rare congenital anomaly, usually found out by continuous precordial heart murmur. Occasionally this disease is found by routine evaluation of cardiac echocardiography or coronary angiography Usually, patients have non specific ECG changes, also less symptom even if significant L-R shunt. In the point of surgical management of CAVF, direct ligation of fistula is usually difficult by the anatomy of fistula vessels. Thus, closure of drainage site was performed under the cardiopulmonary bypass. Thus, the most surgical management is performed under the non beating heart, that cause of difficulties of simultaneous evaluation of coronary artery perfusion. Complications are not rare, such as myocardial infarction, cardiac tamponade, residual shunt, atrial or ventricular arrhythmias after the surgery were reported. The most benefit of interventional procedure of CAVF is the possibility of simultaneous evaluation of coronary perfusion. Additionally, repeat or staged procedure can be possible, CP bypass is not required in the interventional procedure. These can be contributed to reduce the complications. Currently, catheter intervention of CAVF is performed by coil, detachable balloon, Amplatzer duct occluder or vascular plug. The benefit of coil occlusion of CAVF is 1) small arterial access, 2) excellent flexibility of coil itself and delivery system, 3) easy repositioning, 4) easy retrieve and 5) availability of various device size. Procedure is performed under the fully hepalinazation (ACT >200 sec.). Selective coronary angiography should be performed by several different projections. After that, test occlusion of distal fistula using small occlusion balloon or regular Berman catheter is performed to confirm whether ischemic changes are produced by occlusion of fistula or not. After the confirmation of no producing ischemic change by test occlusion, coil or other device implantation is performed. After the deployment, repeated coronary angiography is performed to check the device position and presence of residual leakage. Indication of catheter intervention for CAVF has been considered as same as surgical indication. However, under the recent development of interventional procedure, more preventive indication can be considered. Anticoagulation after the interventional procedure is not standardized. Aspirin or sometimes warfarin were used after the procedure, contrary, some investigators follow the patients without anticoagulation. Management of residual shunt is unclear. Long-term outcome after the interventional procedure including natural course of aneurysmal dilatation of coronary artery are not known completely. In conclusion, coil occlusion of coronary AV fistula should be considered as the initial therapeutic option. Optimal timing, anticoagulation, long-term outcome should be re-evaluated under the current interventional results. SPEAKER’S ABSTRACTS November 1, 2006 S5(1/3) Coil occlusion of AV fistula and collateral SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S5(2/3) Stenting of coarctation of aorta Masood Sadiq, M.D. The Children’s Hospital Punjab Institute of Cardiology, Lahore. Pakistan. In our part of the world, coarctation of the aorta (CoA) is not uncommonly missed in early life & patients are diagnosed in adolescence & adult age group usually in the context of investigation for hypertension. It can be managed in different ways depending upon the age of presentation, coarctation morphology, whether the coarctation is native or a recurrence following surgery/angioplasty and the local institutional expertise/results of each type of treatment. Stent implantation is becoming a popular method of treatment in older children and adults but is avoided if at all possible in infants and young children where surgery and balloon angioplasty is preferred treatment. The rationale for stent implantation is that over dilation of the coarctation segment are unnecessary, thus avoiding major transmural tears, while at the same time the stent struts will splint any smaller tears against the aortic wall preventing progressive dissection and aneurysm formation. The acute elastic recoil of the coarctation segment that contributes to a suboptimal initial result and later recoarctation is prevented by stent implantation. In the last decade, stenting has been performed in this age group as an alternative to balloon angioplasty and surgery with good intermediate term results. A particular role for stent implantation is in the situation where aortic valve or root replacement or coronary bypass grafting is needed. Pre-surgical stenting of the coarctation segment removes the need to dissect the coarctation area or try to recover from bypass in the face of an aortic obstruction. The risk of aneurysm formation, dissection, aortic rupture and death, however, has not been eliminated. Excessive stretching of a tight coarctation, however, could still lead to aneurysm formation and rupture so graded dilation, allowing for healing before further dilation. Availability of covered stents may decrease all these complications and has a special place in developing countries where cost is an important issue. Covered stents will be especially of value in our part of the world where staged dilatation of bare stent adds significant cost to the treatment, patient may be lost to the follow up and one off treatment may have to be offered. Co-existent lesions like PDA can be treated in the same setting with no extra cost. This is an additional consideration along with other indications like complex anatomy with tortuous arch, near atretic lesion, long segment coarctation, and transverse arch coarctation, associated aneurysms whether native, after surgery or catheter intervention, Turner syndrome and advanced age with higher risk of dissection or rupture. The most commonly used stent is the balloon dilatable type which allows for further expansion of the stent as child grows or if further dilatation is needed in a critical lesion. Palmaz stainless steel stents (J&J) were the first ones to be used. They have a closed cell PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 design which gives them high radial strength but less flexibility. They can foreshorten significantly (33-50%) if over dilated. Palmaz genesis stent was introduced to overcome some of shortfalls of Palmaz stent and is designed to have low profile. It can be expanded to 18mm while maintaining flexibility and radial force. The intrastent (IT) LD stent (Intratherapeutics, now ev3) was introduced to improve on Palmaz stent. It has an open cell design for better maneuverability. Elastic recoil and distortion were noticed although better suited for curve of the arch. The CP stent (NuMed) is made from heat tempered 90% platinum and 10% Iridium. It can be dilated up to 24mm and is better choice for treating coarctation. To evaluate the use of covered Cheatham-Platinum (CP) stents in the management of native coarctation of the aorta (Co A) in a developing country, 15 covered CP stents were implanted in 13 procedures on 12 patients with native Co A at our institute in the last 3 years. This represents 36% of 33 patients, who underwent stenting of aortic coarctation for native coarctation of the aorta at our center during the same period. These were at risk patients with near atretic lesion, long segment coarctation, transverse arch coarctation, and Turner syndrome, associated PDA and as rescue treatment in a patient with previous stent related dissection. In addition patients with severe lesions who would have needed serial dilatations were offered one off treatment with desired dilatation in one go. One patient had previous bare stent implantation leading to dissection needing 4 covered stents in 2 procedures. Mean patient age and weight were 21( 16) years, range 11-46 yrs and 49( 13) kg, range 35-84kg, respectively. The systolic gradient across the CoA decreased from a mean ( SD) of 45 23mmHg before, to a mean of 3 4mmHg after the procedure (p<0.0001), and the diameter of the CoA increased from 5.7 4mm to 18.1 3.8 mm (p<0.0001). Follow up period was up to 50 months (mean months). All stents were patent and in good position on CT or MRI performed 3-6 months later. In 33% of the patients antihypertensive medication was decreased and in one patient it could be stopped. Our results add to the current limited knowledge about covered stents and these may be used as the therapy of choice in patients with native coarctation of the aorta with severe and complex coarctation of aorta and provide a safe cost effective alternative to conventional stenting in patients likely to need serial dilatations and where they may be lost to follow up. The disadvantages of stenting an aortic wall is that it may alter wall compliance, pressure wave propagation and blood pressure. In young patients additional dilatations will be required to keep pace with growth. Neointimal growth causing mild restenosis may also need additional dilatations. In conclusion, in selected patients with coarctation of the aorta stent implantation is feasible and short and mid term follow up is encouraging. A small incidence of aneurysm formation is inevitable in all forms of treatment. Most procedure related complications can be treated with covered stents. Covered stents may become treatment of choice in adolescents and adults. Long term follow up is needed to address the complications and disadvantages. SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S5(3/3) Interventional Tips and Techniques of PDA Transcatheter Closure Mohammad Omar Galal (Saudi Arabia), M.D., Ph.D., MBA King Faisal Specialist Hospital&RC, Jeddah, Saudi Arabia PDA closure has been introduced over 30 years ago as an alternative to surgical ligation. Today, transcatheter closure is regarded as the first choice of treatment for this disease. There are different types of plugs, occluders and coils devices in clinical use. This intervention is generally regarded as a relatively straightforward procedure, where almost all problems have been resolved. This statement goes for most of the ducts. The different types and sizes of the PDA in relation to the body weight of the patients does need special techniques in order to offer the patient the best and safest outcome. Like for all other procedures, decision-making is the most important part of the intervention. Occasionally few PDA has to be regarded as being not yet amenable for the available devices. Premature babies with large PDA are still problematic. But also older babies with very large ducts can be a challenge. A closure of the window type duct is still a technical brick wall, as it could lead to protrusion of the device into the aorta. Also in the tubular type PDA, the secure anchoring of the available devices can be very difficult if not impossible. In case of the combination of a coarctation and a PDA, possibly the implantation of a covered stent could be the answer. PDA with pulmonary hypertension is another confront for the clinician. In this session the different techniques and tips for PDA closure will be discussed in an interactive way with the attendees. PCCS 2006 Infective Endocarditis in Pediatric Patients: Thailand Experience Vachara Jamjureeruk, M.D. Bangkok Heart Hospital, Bangkok, Thailand Infective endocarditis in pediatric patient is not a common disease. But, this disease is a serious one resulting in significant morbidity and mortality. From our retrospective, collaborative study (Queen Sirikit's National Institute of Child Health, Pediatric Department, Chulalongkorn University and Pediatric Department, Chiangmai University) from 19892002 had the total of IE in pediatric patients 125 cases. There were 64 boys and 61 girls. The age ranged from 7 days to 16 years old. The most common organisms were Streptococcus (33-50%) and Staphylococcus (16.6-41.5 %). The common presenting symptoms were fever (63-92%), congestive heart failure 19-60 %, splenomegaly 16-39 % and petechiae 8-13 %. The common underlying congenital heart diseases were VSD (20-30%) and Tetralogy of Fallot (7.7-12%). The Echocardiogram was very useful for determining the diagnosis of IE by the detection of vegetation and valvular destruction. The numbers of vegetations ranged from 1 to 4 lesions. One vegetation lesion was the most common finding. The most common vegetation site was at MV 31.2 % and then at the TV 11.2 %.The systemic embolism was correlated with the vegetation size of >/ 1 cm. in diameter especially at the left side of the heart. The complication rate was 63 %. The most common complications were congestive heart failure (22%) and brain complication (18%). The overall mortality rate was 19 %. Antibiotics were the mainstay of the treatment. Appropriate choice, adequate dosage with sufficient length of treatment were needed to eradicate the organism. Penicillin or ampicillin with gentamycin were the common antibiotics used at that period and the duration of treatment was 4-6 weeks. In some severe cases, Cloxacillin, Vancomycin and third generation cephalosporin had to be added. Due to IE in pediatric patient is one of the serious disease resulting in significant morbidity and mortality, the early diagnosis and the appropriate treatment especially antibiotics are very important with cautiousness to look for the complications especially systemic embolism, brain complication and mycotic aneurysm. Both TTE and TEE may produce false –negative results if vegetations are small or have already embolized. The persistent vegetation after systemic embolization, big vegetation (>1 cm.) at the anterior MV or AV, valve perforation or rupture, prosthetic valve dehiscence, new heart block or arrhythmias, large myocardial abscess or heart failure unresponsive to medical therapy are the bad signs of disease which usually need surgical intervention. The CT scan and MRI for detecting vegetation are limited in children due to insufficient breath holding and poor cooperation which some of them needed to be under general anesthesia. But this modality is very important for suspicion of intracranial hemorrhage from rupture of mycotic SPEAKER’S ABSTRACTS November 1, 2006 S6(1/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 aneurysm or embolism . The new Duke's criteria is very useful and applicable to the care of pediatric patients. But, clinical presentations of IE in newborn infants have some differences from children. It is mostly associated with using the invasive techniques to manage neonate with multiple complex medical problems. The morbidity and mortality rate are also high in this age group. The IE also should be aware in unexplained high and prolonged fever pediatric patient despite no definite heart murmur. PCCS 2006 Risk factor for coronary heart disease: management in childhood in developing countries Kalim Uddin Aziz, M.D. Professor (Emiratus) of Paediatric Cardiology National Institute of Cardiovascular Diseases, Pakistan There is evidence to suggest that risk factors (RF) for coronary heart disease (CHD) are present during early childhood and can be detected. These RF track to adulthood so that hypertensive, hypercholesterolemic and obese child to ill became hypertensive, hypercholestrolemic and obese adult .. RF data in Pakistani children population showed 5.3 % prevalence of hypertension, 0.5-2.2% obesity and 9-14% hypercholesterolemia. These RF can be modified and this has formed the basis of early prevention of CHD in the early childhood RF modifiers are physically active life style, molding of daily consumption of calories to the physical activities, modification of diet which involves reduction of fat consumption, substitution of saturated fats with oils, increase consumption of white meat, fish, fruits and vegetables. Various strategies are available for implementing these RF modification recommendations Universal, community based and targeted, which is physician based. Pros and Cons of implementation of these recommendations in the developing countries shall be discussed. SPEAKER’S ABSTRACTS November 1, 2006 S6(2/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S6(3/3) Metabolic syndrome in children Masao Yoshinaga, M.D, Ph.D. National Hospital Organization Kagoshima Cardiovascular & Cancer Center Obesity is a serious problem among young people as well as among adults. The number of preschool children and adolescents who are obese is increasing in both developed and developing countries, and the current evidence suggests that this problem is rapidly worsening. 1) Rapid increase in the prevalence of obesity in Japanese elementary school children.1) As regards the development of obesity in childhood, there appears to be at least three critical periods: fetal; a period of adiposity rebound between ages 4 and 6; and adolescence. Our study showed that a significant risk for development of obesity during the elementary school years (age 6-11 years) applied only to boys from around 1993 onward in our City and applied throughout the study period (1989-2002) in nationwide Japan. 2) Metabolic syndrome in overweight (at risk for obesity) and obese children.2) Japanese obese children were found to have a significantly lower prevalence (17.7%) of metabolic syndrome than US obese adolescents (28.7%, p=0.0014). However, Japanese overweight children have a similar incidence (8.7%) of metabolic syndrome when compared with US overweight adolescents (6.8%). Hyperinsulinemia in females and abdominal obesity in males are characteristic features of individual metabolic syndrome in Japanese children. 3) Emergence of cardiovascular risk factors from mild obesity in children.3) In Asian countries, especially Japan, obesity-associated disorders arise in mildly to moderately obese adults. A tendency toward early development of cardiovascular risk factors from mild obesity was found not only in adults but also in elementary school children. An important feature in boys is that an abrupt worsening of insulin resistance can emerge from both mild and severe obesity. 4) Prevention of mildly obese children from development of more obese condition.4) Two programs began in 1992 in Kagoshima City; first, a program to screen overweight children with obesity-related complications, and second, a program to treat obese children. The treatment program was found to be important because it prevents mildly obese children, who may worsen their obesity indices without the program, from developing more obese condition. 4,5) References 1) Yoshinaga M, et al. Int J Obes, 2004 ;28:494-9. 2) Yoshinaga M, et al. Obes Res, 2005;13:1135-40. 3) Yoshinaga M, et al. Diabetes Care, 2006; 29:1408-10. 4) Yoshinaga M, et al. Prev Med, 2004; 38:172-4. 5) Tanaka S, Yoshinaga M, et al. Circ J, 2005; 69:232-6. PCCS 2006 Dong Man Seo, M.D. Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea SPEAKER’S ABSTRACTS November 1, 2006 LS1(1/3) Value repair in congenital MS/MR SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 LS1(2/3) Mitral and tricuspid valve repair using a Biodegradable Ring in pediatric population Afksendiyos Kalangos, M.D. Clinique de Chirurgic Cardio Vasculaire, HCUG, Geneva, Switzerland PCCS 2006 The Kalangos Ring in pediatric valve repair Mohamad Azhari Yakub, M.D. National Heart Institute, Kuala Lumpur, Malaysia SPEAKER’S ABSTRACTS November 1, 2006 LS1(3/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S7(1/3) The Role of Multislice Computed Tomography in the Diagnosis of Congenital Heart Disease William C.L. Yip, M.D. National University Hospital Singapore Baby & Child Clinic Currently the most important tool for diagnostic evaluation of congenital heart disease (CHD) is echocardiography. In experienced hands most of the congenital heart defects in infants and young children can be delineated with confidence. Indeed many of them can be surgically corrected without the need of cardiac catheterization. However, crucial information of certain cardiac anatomy, like extracardiac structures, systemic and pulmonary veins and surgical shunts, may not be adequately imaged when there is poor or no echo-window, especially in older children and adults. Although computed tomography has been occasionally utilized to study the anatomy of the heart, principally by cross sectional imaging for some time, the role of the recently available multislice computed tomography (MSCT) in the evaluation of CHD has not been well defined. This paper is not a report of comparative evaluation of MSCT and other modalities of imaging. Rather, it aims to demonstrate the ability of MSCT to display specific cardiac anatomy, deemed difficult to or suboptimally imaged by echocardiography. MSCT enables not only examination of the heart in two-dimension in multiple number of sub-millimeter thin slices in any selected plane, but also display of astonishingly accurate reconstructed three-dimensional structures, reminiscent of the gross specimen of the heart. Advancement in computer technology also allows differential colour-coding, isolation of structures of interest and rotational viewing, enabling the study of the heart without actually cutting and touching it, in a manner never possible before. Moreover, complex CHD is not infrequently associated with tracheobronchial / pulmonary anomalies, which can be displayed in exquisite clarity by MSCT. Finally the currently available 64slice computed tomography allows rapid acquisition of the data set in less than 5 seconds, thus minimizing patient’s discomfort and saving time, compared to cardiac catheterization. Hence MSCT definitely has an useful role in the diagnostic evaluation of CHD. The unique role of MSCT in the diagnosis of CHD can be summarized below: 1. Demonstration of extracardiac structures, like aortic arch anomalies, peripheral pulmonary artery anomalies, systemic and pulmonary vein anomalies, and coronary artery anomalies 2. Display of surgical shunts 3. Delineation of connectional and spatial anomalies, and 4. Demonstration of airway structures and tracheobronchial/pulmonary anomalies associated with CHD PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 For diagnosis of complex CHD by MSCT, one has to identify first the three cardiac segments, viz. the morphologic right and left atria and ventricles, and the great arteries, namely, aorta and pulmonary artery. This is achieved by identification of the respective typical morphological atrial appendages to establish the atrial situs. The definition of the atrial situs can be further collaborated by the display of morphology of the bronchial trees and lungs. The characteristic trabecular morphology of the respective ventricles can also be accurately demonstrated by MSCT, thus confidently differentiating the morphologic left from the right ventricle. The branches of aorta and the peripheral pulmonary arteries can be identified with ease from their respective origin. With experience, the systemic and pulmonary venous structures can also be demonstrated without much difficulty. The next step is to display the connection of the three segments of the heart and the venous drainage to the respective atrial chamber. The connectional and spatial relationship of the various components of the heart is best demonstrated by multiple two-dimensional slices of the heart in different planes. Finally the associated structural lesions (like septal defects and valvar stenosis or atresia) and variation of the size of the components of the heart (like hypoplasia of ventricular chamber and aortic arch hypoplasia) can be clearly demonstrated by MSCT. In summary, because of the unique features of MSCT, it enables cardiologists to image accurately extra-cardiac structural defects and complex CHD which is frequently associated with complicated connectional and spatial abnormality. This is especially important in older children and adults, where echocardiography is frequently handicapped by poor or no echo-window and by its inability to image clearly the tracheobronchial tree and the extracardiac structures. However, in view of the radiation exposure, MSCT should only be carried out when the anatomy cannot be adequately displayed by echocardiography and simultaneous cardiac catheterization is not deemed necessary in the same occasion, in order to minimize the radiation risk to patients. SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S7(2/3) Advance of cardiac MRI in the evaluation of CHD Suvipaporn Siripornpitak, M.D. Department of Radiology, Ramathibody Hospital, Mahidol University, Bangkok, Thailand PCCS 2006 3D and 4D echo: Does it matter? David J. Sahn, M.D. Professor of Pediatrics, Diagnostic Radiology, Obstetrics & Gynecology and Biomedical Engineering Director, OHSU Interdisciplinary Program in Cardiac Imaging L608, Pediatric Cardiology Oregon Health & Science University SPEAKER’S ABSTRACTS November 1, 2006 S7(3/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S8(1/3) Ventricular Assist Devices (VAD) Anthony C. Chang, M.D., MBA CHOC Heart Institute Children’s Hospital of Orange County “See Chang, A. C. and E. D. McKenzie (2005). “Mechanical cardiopulmonary support in children and young adults: extracorporeal membrane oxygenation, ventricular assist devices, and long-term support devices. “ Pediatr Cardiol 26(1): 2-28.” PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 S8(2/3) Post – operative ECLS Laurance L. Lequier, M.D., FRCP (C) Director of ELCS Program, Strolley’s Children Hospital, Canada SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S8(3/3) Organization of ECLS Team in Medical Center and the Multi-application Yih Sharng Chen (Taiwan), M.D., Ph.D. National Taiwan University Hospital, Taipei, Taiwan Extracorporeal life support (ECLS), also called extracorporeal membrane oxygenation (ECMO), had been widely applied in the neonatal pulmonary disease with excellent and stable outcome. However, ECLS for neonatal pulmonary disease is gradually decreased because of the recent advancement of pulmonary physiology and ventilator. ECLS for Cardiac support is gradually accepted internationally and the result is improving. The patient group is also extended from neonate to pediatric group, and even to adult patients. Organization of ECLS team is essential for a medical center to start a steady ECLS program. Physicians, including pediatric ICUist, pulmonologist, cardiologist and surgeons (cardiovascular surgeons is more suitable), special technicians and nurses should recruited. Centralization of instrumentation, simplification of the equipment and regular member training and education are important issue to maintain improvement of the ECLS program. However, adequate case for ECLS (the economic scale) is important to keep the team economic balance. With the improvement in ECLS, it has been applied not only in postcardiotomy shock, but also applied in patients under resuscitation and cardiac massage, non-heartbeating donation. Our data of ECLS application in CPR had 33% survival in adult and 40% survival in pediatric group. In pediatric group, the patients with isolated cardiac disease had survival rate of 47% and better outcome in those with other medical conditions had survival of 25%. The non-survivors had a higher serum lactate levels, longer duration of cardiopulmonary resuscitation and presence of renal failure after ECPR. 80% of the survivors had normal neurological function. We also applied ECLS as screening tool to select suitable candidate for VAD implant. It is controversial in VAD or ECLS selection in the early stage of collapse. But the selection of the proper mechanical support is location/nation specific. It depends on the different local sources in different nations or areas. ECLS is improving its selection criteria and it outcome. PCCS 2006 Lateral tunnel vs extracardiac Fontan: Which is the best? Thomas L. Spray, M.D. Children’s Hospital of Philadelphia, PA, USA SPEAKER’S ABSTRACTS November 1, 2006 S9(1/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S9(2/3) Fontan in heterotaxy syndrome Hiromi Kurosawa, M.D. Tokyo Women’s Medical University, Tokyo, Japan PCCS 2006 Alternatives for unsuitable Fontan Roger B. B. Mee, M.D. Chairman Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic. (Retired) For an unseptatable heart the Fontan type procedure offers the most efficient circuit, in theory, but when performed on less than suitable patients, the result is unpleasant. Therefore, considerable thought, based on good data, needs to be exercised before tracking a patient towards a Fontan, and if so tracked, before the final decision to complete the Fontan. The Bidirectional Cavo Pulmonary Shunt has been a useful intermediate step but, again, should not be used automatically. In less than suitable cases, there are alternatives which will be associated with a better quality of life. There are factors which may suggest unsuitability for a Fontan, but if they are correctable, an at least average Fontan may ensue. Static Fontan criteria have been established (Fontan & Baudet) and variously challenged. It may be more useful to work with a list of prospective criteria. Whatever the management strategy, the basic principles of Fontan tracking are based on early diagnosis. Preliminary palliations should be designed to preserve the entire pulmonary circuit and avoid prolonged volume and pressure overload on the ventricular mass. The most common errors in management decision making are based on erroneous calculation of total resistance to forward blood flow through the pulmonary circuit, and on the shallowness of “wishful thinking”. Sometimes, interim repair of fixable defects, may result in a patient who proves more suitable for a Fontan when reassessed a year or two later. If it is determined that a given patient will have a less than average Fontan result, a better quality of life may be offered by procedures providing controlled pulmonary blood flow in the presence of venous admixing. The 1 ? ventricle repair offers an alternative but is usually only usable in patients who could have had a Fontan procedure. For non-septatable hearts, all available procedures, including heart transplantation, offer a palliation to patients who won’t have a long life expectancy. SPEAKER’S ABSTRACTS November 1, 2006 S9(3/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S10(1/3) Marfan syndrome Heung Jae Lee, M.D. Professor of Pediatrics, Sungkyunkwan University School of Medicine Samsung Medical Center, Seoul, Korea The Marfan syndrome (MFS) is an autosomal dominant disorder of connective tissue characterized by involvement of three major systems (skeletal, ocular, and cardiovascular) due to alteration in microfibrils, but with highly variable clinical manifestations. Mutations in the gene for fibrillin-1 (FBN1) at 15q21.1 was found to cause Marfan syndrome in 1991, and in 2004 TGFBR2 at 3p24.1 was newly identified as the Marfan syndrome type II gene. More than 500 FBN1 mutations have been found to date, and tentative genotype - phenotype correlations have emerged. FBN1 mutations have been found not only in MFS, but also in a range of connective tissue disorders collectively termed ‘fibrillinopathies’ ranging from mild form of isolated ectopia lentis, to severe disorders including neonatal MFS, which generally leads to death in infancy. TGFBR2 mutations are still limited, however, in 2005 were also reported to cause a new aneurysm syndrome. This review intends to provide an overview of clinical overview of MFS and the mutations found in MFS. The review will includes the clinical characteristics of ca 230 MFS patients listed in database of the Marfan clinic in Samsung Medical Center(SMC), and will include the results of our mutation analysis of FBN-1 gene in 54 Koreans with MFS(Yoo, Lee & Ki) From May 2003 to July 2006, a total of 54 Korean patients were referred to SMC for FBN1 gene analysis. Among them, 23 patients fulfilled or possibly meet the diagnostic criteria of MFS by Ghent criteria (Group I) but 9 patients did not (Group II). Remaining 22 patients could not be evaluated due to lack of information (Group III). All patient’s genomic DNA was extracted, amplified and sequenced for whole exons and their flanking intronic regions of the FBN1 gene. In Group I, 18 out of 23 patients (78.3%) had the FBN1 gene mutations but none in Group II had the mutation. In Group III, FBN1 gene mutations were found in 10 patients (35.5%). Among 29 FBN1 mutations identified in the present study, 22 were novel and 7 were previously reported mutations. Sixty-two percent of the all mutations (18/29) occurred in cbEGF-like modules and 44.8% (13/29) were either nonsense or frameshift mutations that led to a premature termination codon. In line with previous reports, classical or suspected MFS patients showed higher probability of carrying FBN1 gene mutation but none had the mutation who did not fulfill the Ghent criteria. Therefore, careful evaluation of any patient with suspected MFS according to the Ghent criteria is important for predicting positive result on FBN1 gene testing and the mutation analysis of FBN1 gene is very useful for confirmatory diagnosis and genetic counseling for MFS. PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 S10(2/3) Genetic basis of cardiac arrhythmias Chun-Wei Lu, M.D. SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S10(3/3) 22q11 deletion In-Sook Park, M.D. Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea PCCS 2006 Novel ECHO parameter for cardiac functional evaluation David Sahn, M.D. Oregon Health Sciences University, Portland, OR, USA SPEAKER’S ABSTRACTS November 1, 2006 S11(1/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S11(2/3) Tele-echo for congenital heart disease: Our experience in India Sunita Maheshwari, M.D. Consultant Pediatric Cardiologist Narayana Hrudayalaya, Bangalore India Diagnosis is key! Without an adequate diagnosis, a child with heart disease may never reach a center that can treat the child, either medically or surgically. Although there are several internists and adult cardiologists in remote India there is a severe dearth of Pediatric Cardiologists or any trained personnel who can do or interpret a Pediatric echo. Even if semi-trained personnel can be deployed in remote areas the high cost of establishing a tele medicine facility may be a deterrent. Over the past 2 years we have, through trial and error, established low cost tele echo solutions for remote India. Technicians or internists/pediatricians spent 2-4 weeks at our tertiary care center learning the basics of Pediatric echo. On return we linked them to our center either via a basic setup which included a video camera or more recently via a locally created software that costs 1500$ as a one time setup charge. Tele-echo with remote sites with the interpreting cardiologist being at our tertiary center in Bangalore was performed on more than 1200 children. Of these children over 350 came to the tertiary center and got operated. The diagnosis via tele-echo was accurate enough to refer straight to the operating room in most patients; however, in a minority of the patients, some changes in the echo diagnosis on site changed the eventual surgical management. The advantage of tele-echo for use in remote areas of Asia are 1) an accurate diagnosis can be made on a child with suspected heart disease and 2) the management strategy can be discussed with the treating doctor and patient before the patient travels long distances to a tertiary center. Thus, high quality pediatric cardiac care can begin to be delivered to children who otherwise would not have access to a diagnosis, let alone treatment. PCCS 2006 Real Time Three-dimensional Transthoracic Echocardiography in Congenital Heart Disease Guo-Ying Huang, M.D. Pediatric Heart Center, Children’s Hospital of Fudan University, Shanghai 200032, China Abstract: A series of studies have shown that transthoracic real-time three-dimensional echocardiography (RT3DE) is able to provide better information on spatial recognition of the VSD and ASD and their relationship to the surrounding anatomic structures as compared with color Doppler echocardiography, and it may become an optimal tool for clinical diagnosis in congenital cardiac septal defects. More recently, the reliability and advantage of RT3DE has been used to evaluate complex congenital heart disease in our center. Fifty patients with complex CHD were examined by RT-3DE with modes of Live 3DE and Full Volume. Ten sectional volumetric views and Van Praagh sequential segmental approach were introduced to reveal the pathological morphology of the hearts, which were compared with the findings of two-dimensional echocardiography, angiography and cardiac surgery. The results showed that 190 image acquisitions of Full Volume were performed at several acoustic windows including subcostal, apical and parasternal regions. Among them, 94.2% (179/190) of image acquisitions were successful. Most sectional volumetric views could be clearly displayed in 92.6% of the successful image acquisitions. However, sectional volumetric views could not be clearly displayed in 7.4%, which was mainly due to poor perspective conditions of examination location, improper instrument multi-parameter setting and insufficient information of whole heart captured in Full Volume acquisitions. As compared with surgical findings and angiography, RT-3DE made correction to the diagnoses in 2 cases including one with corrected transposition of the great arteries and the other with single atrium and mitral cleft. The diagnoses initially made by 2DE for these 2 patients were complete transposition of the great arteries and complete atrio-ventricular septal defect. We concluded that RT-3DE can clearly display the pathological morphology of complex CHD by using sectional volumetric views combined with sequential segmental approach through providing more spatial informative cardiovascular structures. Key Words: echocardiography; real time; three dimensional imaging; congenital heart disease SPEAKER’S ABSTRACTS November 1, 2006 S11(3/3) SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S12(1/2) Case presentation from SCMC Zhuoming XU, M.D., Ph.D. Cardiac Intensive Care Unit, Shanghai Children’s Medical Center, Shanghai, China PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 S12(2/2) Case presentation from SCH Laurance L. Lequier, M.D. Strolley’s Children Hospital, Canada SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S13(1/3) Childhood cardiomyopathy: Experience in Australia James L. Wilkinson, M.D. Royal Melbourne Hospital, Melbourne, Australia PCCS 2006 SPEAKER’S ABSTRACTS November 1, 2006 S13(2/3) Cell therapy Christoff STamm, M.D. Deutsches Herzzentrum Berlin, Berlin, Germany SPEAKER’S ABSTRACTS November 1, 2006 PCCS 2006 S13(3/3) Heart transplantation Yih-Sharng Chen, M.D., Ph.D. National Taiwan University Hospital, Taipei, Taiwan Cardiomyopathy continues to be a unsolved and unclear topic in heart failure. Several advancements had been approached with improvement. Early detection of the failing heart with more accurate tool, BNP, MRI and tissue Doppler, help physicians to intervene the early stage of heart failure before the failure loses control. The improvement in pharmacology and exercise physiology also extended the survival of chronic failure and the quality of the life. Pediatric heart transplantation has undergone major changes over the past two decades, marked by a substantial improvement in survival, ultimate the ABO-incompatible infant donors, and enhancement in quality of life for transplant recipients. Actuarial survival has improved substantially in the last decade. Due to the shortage of pediatric donor hearts in Asia, various biological and mechanical bridge methods had been used to prolong waiting patients’ survival. There are now new left and right ventricular assist devices that are suitable for use in infants as a bridge to transplantation. New immunosuppressive agents have reduced the risk of rejection while minimizing side effects and strategies to reduce the risk of graft coronary disease are beginning to show promise. Finally, long-term survival for children after heart transplant is improving to better quality of life. PCCS 2006 S14(1/3) Surgical repair: How to avoid late complication SPEAKER’S ABSTRACTS November 2, 2006 Shunji Sano, M.D. Okayama University Graduate School of Medicine and Dentistry, Okayama, Japan PCCS 2006 S14(2/3) SPEAKER’S ABSTRACTS November 2, 2006 Physiologic determinant of PR Andrew N. Redington, M.D. Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada PCCS 2006 S14(3/3) The role of the evaluation of PR/RV function post TOF repair In patients with TOF repair, long-term postsurgical outcome depends on chronic PR, RV dilatation, and ventricular dysfunction. Cardiac MRI is a well-established diagnostic method (the standard of reference) for quantification of PR fraction and evaluation of RV function, and thereby it may be helpful to determine optimal timing of intervention for preventing deterioration of cardiac function and improving long-term outcome during serial postoperative follow-up of this cohort. ECG-gated phase-contrast imaging is used for calculation of PR fraction and ECG-gated balanced steady state free precession (SSFP) imaging provides end-diastolic and endsystolic volumes, ejection fraction, and mass of RV. Although both MR techniques are quite accurate and reproducible, several limitations and pitfalls are worth considering. For PR fraction, data obtained from the main pulmonary artery may not be accurate because of its short length and motion. In such case, we can rely on data calculated from both branch pulmonary arteries. To overcome this limitation, a vessel-tracking technique should be employed to phase-contrast imaging in the future. The same PR fraction may have different clinical impacts and, therefore, it should be interpreted with other hemodynamic parameters. It is not easy in defining paravalvuar (the pulmonary and tricuspid valves) areas of RV, which is a main source of error in RV volume measurement. Cross-reference between short- and long-axis cine imaging is quite helpful to reduce this error. Better segmentation between myocardium and blood may eventually increase the accuracy of MR evaluation of RV function. Exclusion of RVOT aneurysm and correction of pulmonary regurgitation are other unsolved problems in the evaluation of RV function. Comprehensive analysis of hemodynamic events occurred in RV and MR tissue Doppler evaluation will enhance our understanding of substrates in MR evaluation of post TOF. Further studies are necessary to establish more clear guidelines of PR and RV function provided by MRI as to when patients with TOF repair should be treated. SPEAKER’S ABSTRACTS November 2, 2006 Hyun Woo Goo, M.D. Department of Radiology University of Ulsan College of Medicine, Asan Medical Center PCCS 2006 S15(1/3) SPEAKER’S ABSTRACTS November 2, 2006 Evolution of Acute Rheumatic Fever (ARF) presentation: Does the Jones Criteria work well in high prevalence countries? Nigel Wilson, M.D. Pediatric Cardiologist Green Lane Hospital Clinical Services Starship Hospital Auckland, New Zealand The risk of ARF after the first attack of group A streptoccoci is approximately 0.33%, but with subsequent infection this risk rises to 25-75%. In addition, those who suffer carditis during their initial attack are significantly more likely to develop further carditis with subsequent streptococcal throat infections. Secondary penicillin prophylaxis reduces the incidence of recurrent rheumatic fever, the need for hospitalisation and cardiac surgery. Hence it is important not to under-diagnose ARF especially in developing countries. There is no single symptom, sign, or laboratory test that is diagnostic for ARF. The Jones criteria for the diagnosis of ARF were introduced in 1944. The criteria divide the clinical features of ARF into major and minor manifestations, based on their prevalence and specificity. The Jones criteria have been periodically modified and updated by the American Heart Association. The 1992 update is currently the most widely used and quoted version. Each change to the Jones criteria was made to improve specificity at the expense of sensitivity, largely in response to the falling incidence of ARF in the USA. As a result, the criteria may not be sensitive enough to pick up disease in high incidence populations in whom the consequences of under-diagnosis are likely to be greater than those of over-diagnosis. Moreover, the pre-test probability for the diagnosis of ARF varies according to location and ethnicity e.g. a child with fever and arthritis is more likely to have ARF in a region with high ARF incidence compared to a region with low ARF incidence, similarly indigenous patients in regions with high ARF are more likely to have ARF than non-indigenous patients in the same region. Recent data from New Zealand shows the incidence of subclinical or echocardiographic only carditis is seen in 30-35% of patients but influences the diagnosis in only 10% of cases with ARF. In addition 2nd and 3rd degree heart block in ARF is not uncommon, has benign prognosis, but is of diagnostic significance. Studies from India, Australia and New Zealand reveal that mono-arthritis can be a frequent presentation in ARF as well as classical migratory polyarthritis or polyarthralgia. The main modifications made to the Jones 1992 criteria for the recent New Zealand guidelines (http://www.heartfoundation.org.nz) and Australian guidelines (http:// www.heartfoundation.com.au) are 1) echocardiographic evidence of subclinical carditis as a major criterion 2) monoarthritis can be a major criterion especially when there is a history of PCCS 2006 SPEAKER’S ABSTRACTS November 2, 2006 non-steroidal anti-inflammatory use that is likely to have aborted migratory polyarthritis Conclusion. The Jones criteria still forms the basis for diagnosis of ARF but in countries with high prevalence of ARF experienced clinicians are expanding the criteria to avoid under diagnosis. PCCS 2006 S15(2/3) SPEAKER’S ABSTRACTS November 2, 2006 The use of Echocardiography in Acute Rheumatic Fever. Manat Panamonta, M.D. Khon-Kaen University, Khon-Kaen, Thailand Acute rheumatic fever (ARF) is an inflammatory syndrome which follows group A beta-hemolytic streptococcal infection of the throat. ARF consists of clinical manifestations including carditis, arthritis, Sydenham’s chorea, subcutaneous nodule and erythema marginatum. However, carditis is the most important manifestation of ARF because it can lead to permanent rheumatic heart disease and death. Diagnosis of carditis in ARF is generally based on auscultatory findings of either mitral regurgitation or aortic regurgitation or both. The reported prevalences of carditis in ARF range from 41-93%. Currently, color Doppler echocardiography (CD Echo) can detect all audible valvular regurgitation and probably can differrentiate the unheard pathological valvular regurgitation so called subclinical carditis (SCC) from functional or physiological flow disturbance. WHO criteria are widely accepted for echocardiographic diagnosis of SCC including: the jet should be > 1 cm in length, seen in at least 2 planes, have a peak velocity > 2.5 m/sec, and persist throughout systole(mitral valve) or diastole(aortic valve). To date, the reported prevalences of SCC in ARF ranged from 0% (in one study) to 53% in 23 reports. In addition, the data of these reports also suggested that approximately half of the patients diagnosed with SCC at the time of ARF had persistence or deterioration of the carditis over the two-year follow-up. Therefore, it became apparent that CD echo has been proven to be a useful tool in the early diagnosis of rheumatic carditis and for the follow-up of rheumatic heart disease. The great concern is how long of the prophylactic duration for a patient with SCC. Since the duration of secondary prophylaxis depends on the risk of ARF recurrence including the presence of clinical (auscultatory) evidence of carditis. According to the WHO recommendation, the duration of secondary prophylaxis should be continued beyond 5 years after the last attack of ARF or until 18 years of age (whichever is longer) in a patient without proven carditis. However, the evolution of valve damage during a recurrence, in a patient who did not have apparent carditis in the initial attack of ARF, has been a subject of debate. It has long been known that a patient who has no apparent carditis in the initial attack of ARF is likely to remain free of carditis during a recurrence. However, the study from Thailand revealed that rheumatic recurrence can develop new valve damage evident by echocardiography for the patients with initially SCC. Longer duration of secondary prophylaxis is recommended to prevent a recurrence of rheumatic fever in the patient with SCC. PCCS 2006 S15(3/3) RHD after 30 years of benzathine penicillin SPEAKER’S ABSTRACTS November 2, 2006 Hung Chi Lue, M.D. National Taiwan University, Taiwan PCCS 2006 P2(1/2) SPEAKER’S ABSTRACTS November 2, 2006 Tissue Engineering in CHD Hiromi Kurosawa, M.D. Tokyo Women’s Medical Cernter, Tokyo, Japan PCCS 2006 P2(2/2) Advanced ultrasound methods for procedural guidance in non-invasive cardiovascular imaging SPEAKER’S ABSTRACTS November 2, 2006 David Sahn M.D. Oregon Health Sciences University, Portland, OR, USA PCCS 2006 S16(1/3) SPEAKER’S ABSTRACTS November 2, 2006 Basic mechanism and treatments of heart failure in children Makoto Nakazawa, M.D. Sounthern TOHOKU General Hospital, Sounthern TOHOKU Research Institute, Fukushima, Japan PCCS 2006 S16(2/3) LCOS in CICU without mechanical support : What is possible ? Mechanical support is often considered a routine tool in the PCICU and is available for rapid deployment whenever needed in most developed countries. In India, despite rapid industrialization over the last decade pediatric cardiac care is largely provided by non governmental institutions. Our public health care policy does not cover expense for pediatric cardiac care and health insurance excludes “all birth defects”. Thus, the prohibitive costs of mechanical support has to be largely borne by the family. Most instances of severe refractory low cardiac output state (LCOS) where mechanical support may be useful often occur in infants and children whose families are least able to incur such expenses. Thus, in our country alternative, less expensive modalities assume increasing importance and often need to be speedily employed. The author’s experience largely constitutes postoperative management of severe LCOS without mechanical support. Even though progressive low cardiac output states occur after cardiac surgery, appropriate anticipation, early identification and aggressive management has been shown to minimize the need for mechanical support. Most pediatric cardiac centers report a < 2% need for ECMO or other mechanical support in infants and children after cardiac surgery with this approach. Thus, our current multipronged approach is highly proactive and focuses greatly on simple, easily affordable measures to diminish requirement of mechanical support in high risk surgeries. Intraoperative measures : This commences with various modalities to minimize cardiopulmonary bypass mediated injury by prophylactic intraoperative corticosteroid therapy combined with modified ultrafiltration to remove inflammatory mediators. Intraoperative ECHOs are done to finetune and perfect surgical repairs. Surgical strategies in selected high risk instances are currently routine practice. Deferred sternal closure in situations of late presentation with very poor left ventricular function or prolonged cardiopulmonary bypass times in complex repairs is “no longer dreaded”. Complications related to deferred sternal closure like wound infection is unusual and we have thus far had no instance of mediastinitis. Similarly creation of right to left shunts to decompress the right ventricle in situations of severe pulmonary hypertension ( hypertensive VSDs, hypertensive multiple shunts) , selected cases of tetralogy of Fallot, truncus arteriosus) is a carefully planned and frequently implemented surgical practice. LCOS protocol : Postoperatively we follow a meticulous approach for early diagnosis of LCOS and deteriorating cardiac output. Clinical, biochemical and hemodynamic parameters are vigilantly followed and monitored so that vasoactive therapy can be titrated and SPEAKER’S ABSTRACTS November 2, 2006 Pavathi Iyer, M.D. Pediatric & Congenital Heart Surgery, Escorts Heart Institute and Research Center, Okhla Road, New Delhi, India SPEAKER’S ABSTRACTS November 2, 2006 PCCS 2006 finetuned. The approach to ventilator dependence and persistent LCOS is aggressive. Residual structural and physiological issues are looked for assiduously by serial ECHOs with a low threshold for cardiac catheterization. Residual defects are addressed by resurgery and collaterals or anomalous vessels appropriately coiled. Milrinone : In our unit, phosphodiesterase III inhibitors like milrinone is usually a second line drug due to cost constraints. However, in situations where there is high risk of LCOS “prophylactic high dose milrinone” is instituted early. We also have a low threshold to use milrinone if the child is not adequately responding to catecholamines. Significant hypotension is not considered a contraindication to milrinone usage, milrinone being administered along with gentle volume augmentation - titrated carefully to LA pressures. We have occasionally used higher than recommended doses up to 2 mcg/Kg/min in severe left ventricular dysfunction. Hormones : In catecholamine and milrinone resistant LCOS or hypotension, we have a low threshold to use corticosteroids. Cortisosteroids are commenced in physiologic doses and usually an improvement in blood pressures have been observed 12-18 hours later. Our practice is based on the published paper which showed a reduction in epinephrine requirement in critically ill cardiac infants after initiation of steroids (1). Similarly, we routinely monitor thyroid functions in all our patients and thyroid hormones are replenished in the presence of documented deficiency (2). Occasionally, we have administered thyroxine even in those infants with borderline or normal thyroid functions with severe LCOS. The benefit of such thyroxine administration is not yet established. PEEP, IPPV : Harnessing cardiopulmonary interactions appropriately has been very useful in managing very severe LCOS. We have used high PEEP of up to 15-20 mm in severe LCOS with left ventricular failure and pulmonary edema even in the presence of severe hypotension. There has been a fairly dramatic improvement in blood pressures followed by improvement in LA p, peripheral perfusion and biochemical parameters. Infants with severe LV dysfunction are electively supported for a week to 10 days with noninvasive respiratory support following extubation. Similarly recommended strategies of early extubation and shorter inspiratory times are used to improve LCOS in infants with “restrictive physiology” like tetralogy of Fallot (3). Metabolic control : Most malnourished infants have persistently low ionic calcium levels in the range of 0.5-0.8mmol/L following CPB. In infants with LCOS, we run a calcium infusion to restore the ionic calcium homeostasis. In significantly hypocalcemic patients with LCOS, calcium repletion appears to improve mean arterial pressures. More recently glycemic control with insulin administration has been shown to improve morbidity due to LCOS in adults undergoing cardiac surgery (4). Pediatric data is limited but preliminary published data indicates that hyperglycemia is associated with increased mortality and morbidity. In severe LCOS and significant hypotension with no residual structural defects the most useful measures have been a combination of modalities -early initiation of high dose milrinone, calcium infusion, early institution of corticosteroids and continued PCCS 2006 SPEAKER’S ABSTRACTS November 2, 2006 ventilatory support with moderately high PEEP. Management of PAH : Management of severe postoperative pulmonary hypertension and pulmonary hypertensive crises has also been considerably improved largely by refinements of intraoperative and postoperative care with emphasis on avoidance of triggers of pulmonary artery hyeprtension (PAH), improvements in ventilatory strategies, and the use of sildenafil as first line pulmonary vasodilator. We have no experience with arginine vasopressin or newer drugs like nesiritide, levosimendan since they are unavailable in India. With a judicious combination of pharmacological methods, appropriate use of respiratory support and PEEP and attention to calcium homeostasis, it has been possible to achieve acceptable overall survival rates even in instances of severe PAH and LCOS. With a mean basic Aristotle score around 6.8 to 6.9 our current overall 30 day survival has improved to between 98 to 98.5 % over the last 4-5 years. Our current neonatal 30day survival ranges between 89 to 91 % and 31day to 1 year survival ( the sickest group which includes infants of late presentation, severe PAH and severe LV dysfunction) is 97 to 98.5%. In summary, we believe that with attention to detail and refinements of easily available and affordable modalities most instances of severe LCOS can be salvaged without access to mechanical support – a modality that my country can ill afford. References : 1. Shore S, Nelson DP, Pearl JM, Manning PB, Wong H, Shanley TP et al, Usefulness of corticosteroid therapy in decreasing epinephrine requirements in critically ill infants with congenital heart disease. Am J Cardiol 2001, 88;591-594. 2. Bettendorf M, Schmidt KG, Grulich-Henn J, Ulmer HE, Heinrich UE. Triiodothyronine treatment in children after cardiac surgery : a double blind, randomized , placebo controlled study. Lancet 2000, 12; 356(9229):529-534. 3. Cullen S, Shore D, Redington A. Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot. Circulation 1995, 91(6);1782-1789. 4. Van den Berghe G, Wouters P, Weekers F, Verwaest C, Bruyninckk F et al, Intensive insulin therapy in critically ill patients. N Eng J Med 2001, 345 (19);1359-1366. PCCS 2006 S16(3/3) SPEAKER’S ABSTRACTS November 2, 2006 Heart failure in GUCH Andrew N. Redington, M.D. The Hospital for Sick Children, Toronto, Canada PCCS 2006 S17(1/3) Aortic translocation in TGA/VSD/PS SPEAKER’S ABSTRACTS November 2, 2006 Zhi-Wei Xu, M.D. Shanghai Children Medical Center, Shanghai, China PCCS 2006 S17(2/3) SPEAKER’S ABSTRACTS November 2, 2006 TGA with abnormal coronary pattern: tachnique and precaution Thomas L. Spray, M.D. Children’s Hospital of Philadelphia, PA, USA PCCS 2006 S17(3/3) Long-term follow up after Arterial Switch The justification for making the change from the Mustard or Senning procedures to the “Arterial Switch” (Jatene procedure) was the belief that long term follow up would be likely to show much better survival after the latter operation, even if the early mortality, associated with the “learning curve” was substantially higher? This change occurred at a stage when long term follow up after atrial switch operations was limited to about twenty years and follow up after the arterial switch was limited to little more than five years. Thus the pressure to change strategies was based on limited solid evidence and may be viewed as having been a “leap of faith”? Fortunately the early mortality after arterial switch has dropped to levels comparable with, or better than, the best results after atrial switch procedures so that the tragic problem of very high initial mortality, which lead to the “Bristol enquiry” and all its consequences, has largely disappeared. Long term follow up after arterial switch now extends to more than twenty years and after atrial switch to forty years. Late problems after the earlier (atrial switch) procedures included venous pathway obstruction, arrhythmias, right ventricular failure and tricuspid regurgitation. Deterioration and premature death or need for transplant has become a regular phenomenon by middle adult life (e.g. 40 – 50 years old), or sooner. This has largely confirmed the gloomy prognosis that was predicted after the first twenty years of follow up, at the time that the change to arterial switch was being proposed, although a substantial number of patients have had good quality survival, without major problems, through early adult life, with many females going through successful pregnancies without problems. Long term studies of patients after the arterial switch show a different set of problems which include late coronary stenosis or occlusion – fortunately rare, aortic regurgitation which becomes more frequent with longer term survival / follow up but rarely requires intervention within the first twenty years of follow up, pulmonary stenosis (which has become less common with improved surgical technique) and rare arrhythmias. Recent evidence raises the prospect of a predisposition to coronary atheroma and later ischaemic heart disease, but this remains unproven at the present time. Overall the actuarial survival after arterial switch is excellent, with little late mortality during the follow up thus far. SPEAKER’S ABSTRACTS November 2, 2006 James L Wilkinson, Prof., M.D. Honorary Cardiologist, Royal Melbourne Hospital, Melbourne, Australia PCCS 2006 LS2(1/3) SPEAKER’S ABSTRACTS November 2, 2006 Management of Eisenmenger Syndrome (from conventionalto novel approach) Kritvikrom Durongpisitkul, M.D. FACC Pulmonary arterial hypertension (PAH) is a recognized complication of congenital heart disease (CHD) with systemic to pulmonary arterial cardiac shunts. Patients will develop degree of oxygen desaturation. In Thailand, around 15% of patient who routinely underwent cardiac catheterization had pulmonary hypertension and required acute pulmonary vasodilator testing. Eisenmenger physiology (EP) is the most extreme case of the above phenomenon since it is associated with high pulmonary resistances, right to left shunting with suboptimal oxygen saturation, and usually large areas of defects allowing the blood to easily circulate from right to left. The conventional management of this group of patients will be discussed. We also shared the experience of empirical management with various pulmonary vasodilator drugs. Also including is a recent double-blind, randomized, placebocontrolled study (BREATHE-5), patients with Eisenmenger physiology treated with new dual endothelin receptor antagonist (bosentan) which showed a significant decrease in pulmonary vascular resistance (PVRi) (treatment effect -472dyn.sec.cm-5;p=0.04) and a significant improvement in exercise capacity, measured by 6-minute walk distance (6MWD) (treatment effect +34m; p=0.02). PCCS 2006 LS2(2/3) Management of Eisenmenger Syndrome: Current practice SPEAKER’S ABSTRACTS November 2, 2006 Geetha Kandevello, M.D. National Heart Institute, Kuala Lumpur, Malaysia PCCS 2006 LS2(3/3) SPEAKER’S ABSTRACTS November 2, 2006 BREATH 5 Michael A Gatzoulis, M.D. Royal Brompton Hospital and the National Heart & Lung Institute, London, UK PCCS 2006 S18(1/3) Cardiac intensive care in the developing world: Experience in India Cardiac intensive care in developing countries like India is often perceived as daunting largely due to concomitant problems of late presentation, malnutrition and associated infections including various forms of hepatitis. This talk is about our experience of a decade of pediatric cardiac care in an evolving unit, about the problems we faced and the simple and affordable strategies that were developed to deliver “meaningful” pediatric cardiac intensive care. Our unit was started in 1995 in a private hospital and in the initial years we performed 300-350 cases annually with an unacceptable 30 day mortality of 7.73% despite “apparently good surgery”. More than 80% of all deaths occurred in bi-ventricular repairs and nearly 60 % of all deaths were due to “sepsis syndromes”. In 1995, this appeared to be the situation in many other units across the country. A compelling need to improve the situation led to a stringent morbidity and mortality audit coupled with an in-depth study of risk factors. We interacted closely with large units across the world and some staff members went overseas for brief periods of re-training. Simple, innovative and affordable strategies were evolved and implemented – all of which led to a dramatic improvement in outcomes with reduced morbidity and hospital stays. Currently we do over 550 cases annually with a mean Basic Aristotle score of 6.8 to 6.9 and our overall 30 day mortality has improved to between 1.5 to 2.5 % over the last 4-5 years. About 40 % of surgical procedures occur in infants < 1 year with two thirds of all surgeries being in males. Our current neonatal 30day mortality ranges between 9 to 11 % and 31day to 1 year mortality - ( the sickest group which includes infants of late presentation, severe PAH and severe LV dysfunction) is 1.1 to 2%. “Preventable deaths due to sepsis” in biventricular repairs is unusual today. Problems identified and strategies Severe malnutrition : All deaths in large left to right shunts occurred in severely cachectic infants, problems encountered being pan atelectasis following extubation related to very poor chest wall compliance. This invariably led to a cascade of events leading to repeated reintubations, airleaks, sepsis and sepsis syndromes. Simple strategies evolved include “fast tracking and earlier extubation” (rather than elective prolonged ventilation which was our earlier practice), “elective prone nursing” after extubation for better alveolar recruitment and earlier elective recourse to noninvasive ventilatory support. Likewise our nutritional policy has become more aggressive, enteral feeds being initiated on the very 1st po day in the absence of suspected bowel ischemia. SPEAKER’S ABSTRACTS November 2, 2006 Pavathi Iyer, M.D. Pediatric & Congenital Heart Surgery, Escorts Heart Institute and Research Center, Okhla Road, New Delhi, India SPEAKER’S ABSTRACTS November 2, 2006 PCCS 2006 Sepsis (preexisting and iatrogenic): Sepsis was identified as largely due to highly virulent ESBL producers ie third generation cephalosporin resistant gram negative organisms. Stringent surveillance of CICU nursing procedures as well as surveillance cultures of “sick at risk” infants helped early detection of indolent sepsis. Many infants at presentation (~ 40%) were found to have preexisting gram negative sepsis or asymptomatic urinary infection which were then treated. Antibiotic usage was rationalized . Severe sepsis was managed as per the ‘Surviving sepsis campaign’ guidelines (1). Implementation of these measures alone significantly reduced sepsis related morbidity and mortality. Other important practices : After basics were addressed, further practices implemented have been a highly systematic approach to the early diagnosis of low cardiac output state ( LCOS) followed by a more meticulous and aggressive management (2). Management of the post op infant tetralogy is based on a better understanding of “restrictive physiology” and on published recommendations (3). Perioperative care of the late presenting hypercyanotic tetralogy with extensive collaterals has also been considerably refined and our current 30 day mortality in tetralogy has improved to ~1 %. Management of severe postoperative pulmonary hypertension and pulmonary hypertensive crises has been considerably improved by refinements in intraoperative and postoperative care with emphasis on avoidance of triggers of pulmonary artery hypertension (PAH), improved ventilatory strategies and the use of sildenafil as first line pulmonary vasodilator. Persistent postoperative LCOS as well as ventilatory dependence is aggressively investigated by early and serial ECHOs and re-catheterization. Airway abnormalities and diaphragmatic paresis are assiduously looked for and addressed appropriately. We have also learnt the benefits of “shared knowledge and shared expertise” and practise a highly integrated team approach and continue to audit ourselves carefully. Finance and manpower issues : India has witnessed a radical change in pediatric cardiac care over the last decade. Pediatric cardiac surgery is today largely done in non governmental institutions with costs being largely borne by the families. Our public health care policy does not cover expense for pediatric cardiac care and health insurance excludes “all birth defects”. Total cost ranges from 1,800$ to 5,500$ depending on complexity and duration of hospital stay. ICU cost is roughly $ 130 per day ( iNO and milrinone are additional costs). With increased awareness and economic prosperity not only are more families able to afford this but there is increasing parental and familial expectation with focus being not only on intact immediate survival but also a good quality future life with good mainstream integration. There is a huge manpower crunch with nurses and residents forming the backbone of the unit. Our unit has one senior and two junior surgeons, 2 cardiologists, 2 anesthetists and 1 intensivist. There are no nutritionists or respiratory therapists and only a part-time physiotherapist. There is tremendous migration among nurses and residents to the USA and Europe, thus our backbone is a “highly floating one”! Our CICU care is thus shared, with increasing maternal, aunt or grandmother’s involvement in the ICU care. PCCS 2006 SPEAKER’S ABSTRACTS November 2, 2006 Nurse and resident standards are improved by a focus on in-service education– both being encouraged to continuously update themselves by electronic reading apart from regular discussions on ward rounds. Junior medical staff participate in regional and national meetings while seniors go periodically for retraining to overseas units. Due to this approach, despite a largely floating and migrant population “the basal standard of care and awareness” has gradually improved over the last 11 years especially in intensive care – which was the weakest link !. In summary, even though we continue to battle late presentation, malnutrition and overwhelming infections the developing world should not despair, because we believe it is still possible to salvage 97-98% of all surgical procedures despite huge manpower and resource constraints. References : 1. Dellinger PR, Carlet JM, Masur H, Gertach H, Calandra T, Cohen J, et al . Surviving sepsis campaign guidelines for management of severe sepsis and septic shock. Crit Care Med 2004, 32 (3); 858-873. 2. Wessel DL. Managing low cardiac output syndrome after congenital heart surgery. Crit Care Med ( Suppl) 2001, 29 (10); S220-S230. 3. Cullen S, Shore D, Redington A. Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot. Circulation 1995, 91 (6);1782-1789. PCCS 2006 S18(2/3) SPEAKER’S ABSTRACTS November 2, 2006 Cardiac intensive care in developing world: Experience in China Zhuoming XU, M.D. Shanghai Children Medical Center, Shanghai, China PCCS 2006 S18(3/3) Pediatric cardiac intensive care: Experience in Thailand The pediatric cardiac intensive care is a new subspecialty in Thailand. Most critically ill children that have been admitted in the pediatric intensive care unit are being taken care by pediatrician, anesthesiologist, or cardiac surgeon while the pediatric cardiologists are acting as consultants. This is due to the limited number of certificated pediatric critical care specialist. While private hospitals are rapidly growing in numbers, most rural residents still have very limited access to the health care services. Due to this, the Thai government has initiated the universal coverage scheme and the referral system and regionalized policies have been providing chances for the rural area residents to gain access to the appropriate treatments. However, the misdistribution of facilities and manpower including well trained specialist, advance technology, equipments and patient education are still the problems. Most of the sophisticated equipments and treatments are located in the university hospital and private hospital. In the past five years, there is a significant increase in the number of congenital heart operations and they are the major population in cardiac intensive care unit. Most of them need postoperative care. The strategy to decrease mortality rate and cardiac intensive care unit cost may be answered by early extubation strategy, step down unit, well organized intensive care unit and excellent hospital management. We still need to improve our system to make early diagnosis of congenital heart disease, early management of cardiac critical condition, and a good transfer system which may need the transfer teams that consist of specialists to take care of critically ill children. In developing countries with limited budget, the budgets should be regionalized and the discrepancies must be controlled. Everyone needs to pay attention to the balance between technology and limited resources. There are 62 certified pediatric cardiologists and less number for pediatric cardiac surgeons in Thailand. The recruitment of new staffs and keeping them are difficult because of the salary. In the meantime, the holistic approach and patient center care need to be emphasized and extended to all junior doctors who will be practicing in this high work load field. The result of the survey on pediatric cardiac intensive care practice in Thailand will be presented in this symposium session. SPEAKER’S ABSTRACTS November 2, 2006 Yuttapong Wongswadiwat M.D. Queen Sirikit Heart Center of the Northeast Khon Kaen University Khon Kaen, Thailand PCCS 2006 SPEAKER’S ABSTRACTS November 2, 2006 S19(1/3) A STAGED CATHETER-SURGERY APPROACH IN TETRALOGY OF FALLOT WITH PULMONARY ATRESIA AND DIMINUTIVE PULMONARY ARTERIES Seong-Ho Kim, M.D. Halla General Hospital, Jeju, Korea Pulmonary arteries are defined as diminutive when the Nakata index is smaller than 90, or in cases if the continuous pulmonary arteries are supplied by major aortopulmonary collaterals, it is upto 150. Tetralogy of Fallot with pulmonary atresia and diminutive pulmonary arteries is difficult to manage, even though several methods of treatment have been tried. Last year, a study about a multi staged surgical approach was reported from Melbourne(JTCS 2005). Its subtitle was “unifocalization brings no long-term benefits.” Among 60 unifocalized MAPCAs, 26 were thrombosed, and 12 showed stenosis more than 50%. A new collaborative approach-a staged catheter-surgery approach- has been introduced since stenotic pulmonary arteries could be enlarged by balloon dilation. This approach is composed of 4 steps. First, catheterization is performed to define PA anatomy and collateral circulation, and to embolize MAPCAs with coils. Second, surgery of establishing RV to PA continuity with a conduit or outflow patch is performed, and it increases pulmonary flow and allows catheter access to PA. In step 3, catheterization is performed again to redefine PA and collaterals, to dilate peripheral PA stenosis, to embolize collaterals, and to reassess hemodynamics. Finally, surgery is performed to close VSD and to reconstruct RV outflow tract. Rome et al reported in 1993 the preliminary result of this approach was superior to other approaches such as conservative treatment, primary unifocalization or BT shunt. They could achieve complete repair in 10 of 20 patients. I would like to briefly introduce our results of this staged catheter-surgery approach. Eighteen patients (PAI=55 18) who was diagnosed as Tetralogy of Fallot with pulmonary atresia and diminutive pulmonary arteries followed this staged approach. Fifteen of 18 patients(83%) could achieve complete biventricular repair, and 3 patients died - 2 after first surgery, and one after total correction. The final mean pressure ratio of right ventricle to aorta after total correction was 0.63. Establishment of early right ventricle to pulmonary arterial continuity with subsequent pulmonary balloon angioplasty and coil embolization of collaterals allow eventual successful complete repair in 83% of patients. This may be a superior outcome compared with those of previous reports. Nevertheless, long-term follow-up is warranted. PCCS 2006 S19(2/3) From Bench to Clinical Practice: Lesson Learned From PlatinumCoated Nitinol Device During the past 50 years, several devices for transcatheter closure of atrial septal defect (ASD) and patent ductus arteriosus (PDA) have been developed. With the wonderful superelastic and shape memory property of Nitinol, new devices composed of Nitinol, have been designed in the last decade. These new devices have been extremely tolerated and widely used. Although Nitinol alloy has a good biocompatibility, nickel release after Nitinol device implantation has been demonstrated (Ries et al. 2003, Burian et al 2006). In addition, systemic allergic reaction after Nitinol device implantation has also been reported (Fukahara et al. 2003, Vincent et al. 2003). Because of the nickel’s adverse biological effects, including potent allergen, extreme toxicity and possible suspected carcinogen; nickel release phenomenon after Nitinol device implantation in the body should be concerned. Platinum is a biocompatible metal, resist to corrosion and has no allergic reaction. With nanotechnology, namely Nanofusion, layers of platinum atoms can be placed on top of the Nitinol wire for the purpose of preventing nickel release. The ASD and PDA devices were braided from the platinum-coated Nitinol wires and filled with polyester sheaths for enhancement of thrombogenicity. In the swine model, ASD device was successfully implanted to close ASD that was created by transcatheter balloon dilation of patent foramen ovale. The PDA study, designed by occlusion of the subclavian artery with PDA device, also had a successful outcome. The echocardiographic study showed complete closure of the created ASD within an hour after implantation. The PDA device totally occluded the subclavian artery within a few minutes after implantation. The post-mortem study confirmed complete closure of the ASD and the subclavian artery. It also demonstrated complete neoendothelialization over the surface of the ASD device, occurred within 6 weeks after implantation. In the clinical trial, 33 ASD cases was attempted to perform transcatheter closure. Twenty nine patients had successful deployment and all had complete closure within 1 month after implantation. The procedure-related complications included transient brachial plexus injury in 1, transient complete heart block in 1, and transient junctional rhythm in 3 patients. There was no device-related complication during 4-12 months follow-up. The PDA device was successfully deployed in 30 patients. There was no serious procedural complication. Twenty nine of 30 patients had complete closure within 24 hours after the procedure. One patient still had tiny leak at 8 months follow-up. Serum nickel level was studied in 25 ASD cases and 25 PDA cases, before and within 90 days after implantation. There was no significant difference in serum nickel level in both groups before and after implantation. The platinum-coated Nitinol ASD and PDA devices had good results in both animal experiment and clinical trial. The platinum coating can prevent nickel release from Nitinol alloy after device implantation. SPEAKER’S ABSTRACTS November 2, 2006 Pornthep Lertsapcharoen, M.D. Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand PCCS 2006 S19(3/3) SPEAKER’S ABSTRACTS November 2, 2006 PDA stenting: what is the indication and outcome Mazeni Alwi, M.D. Consultant & Head Department of Paediatric Cardiology Institut Jantung Negara (National Heart Institute), Kuala Lumpur, Malaysia PDA stenting is an attractive alternative to conventional shunt surgery in duct dependent congenital heart disease as it avoids thoracotomy and its related problems. With today’s generation of coronary stents which have better profile, flexibility and trackability, PDA stenting may be achieved safely and with considerably less difficulty than previously described. The indications for PDA stenting is exactly the same at Blalock Taussig shunt. In the modern era of cardiac surgery where corrective/definitive surgery for various complex congenital heart diseases is done in late infancy or early childhood, PDA stenting is indicated mainly in duct-dependent lesions chiefly in the neonatal period. PDA stenting is also indicated as adjunct to pulmonary valvotomy in PAIVS or critical PS when the RV is deemed too hypoplastic to support the pulmonary circulation. Apart from indication, it is also pertinent to discuss about the contraindications of PDA stenting. This is chiefly because stenting the PDA tends to accelerate pre-existing branch PA stenosis. Unlike the PDA as an isolated lesion, the ductus in cyanotic heart disease have a remarkable morphologic variability. The ductus tends to arise more proximally under the aortic arch, giving rise to a vertical PDA or occasionally it may arise from the subclavian artery. It also tends to be long and sometimes very tortuous, rendering stent implantation technically impossible. The ductus in these patients may also insert onto one of the branch pulmonary arteries with some stenosis at the site of insertion (pulmonary coarctation). The ductus in TOF with PA tend to exhibit these morphologic features and to a lesser degree TGA-VSD-PS and the more complex forms of univentricular hearts. The presence of pre-existing branch PA stenosis at the site of PDA insertion should be considered a contra-indication for PDA stenting, especially in those destined for the Fontan track. PDAs that are long and very tortuous is also a contraindication for technical reasons. PDAs that are larger than 3.5 mm should also be considered contraindication if small coronary stents are to be used because of the likelihood of stent migration. PDA stenting is effective maintaining ductal patency and provides sufficient palliation until the definitive surgery in the majority of cases. However the durability of stent patency is considerably less than Blalock Taussig shunt due to rapid neo-intimal proliferation. This may require a palliative Blalock Taussig shunt in a small number of cases. The major complication of PDA stenting is acute thrombosis but this is uncommon. Haemodynamic instability is often transient. ICU and hospital stay tends to be more PCCS 2006 SPEAKER’S ABSTRACTS November 2, 2006 favourable compared to Blalock Taussig shunt. In summary, PDA stenting in neonatal duct-dependent cyanotic congenital heart disease is a preferred choice of first palliation. This is however contraindicated in those with branch pulmonary artery stenosis. PDA stenting may also be indicated in certain cases of pulmonary atresia with intact septum at the time of transcatheter valvotomy. PDA stenting is safe and effective in providing short-term palliation. The post procedural morbidity, ICU and hospital stay compare favourably to conventional surgical shunt. PCCS 2006 S20(1/3) SPEAKER’S ABSTRACTS November 2, 2006 Life-long concern in CHD: Experience of the GUCH clinic in Asia-Pacific James Yip, M.D. National University Hospital, Singapore An estimated 11,000 adults with congenital heart defects (ACHD) live in Singapore. At least half, face the prospect of complications, reoperation and premature/sudden death. Their numbers are growing. Experts in the field know that health risks can be reduced and outcomes improved if co-ordinated care by qualified and informed health care professionals were available in the country. Pediatric care for patients with CHD, over the past 25 years, has been so outstanding that as many as 85% of these children now survive into adulthood. Given these successes, it has long been apparent that a “new group of adult patients” was going to appear in Singapore. Remarkably and inexplicably, no provision was made to care for them after they “graduated” from the pediatric units. Many were simply lost to follow-up as adolescents or young adults. Most had not been educated about their expected future health issues, or their responsibilities for their own care. This has led to a marked lack of access to qualified and informed cardiology and primary care specialists. Most adult cardiologists are not qualified to care for many of these patients according to leaders in CHD. Many paediatric cardiologist are similarly not trained to care for adult problems like ischemic heart disease, arrhythmias and issues of pregnancy. It is known that the outcome of CHD patients cared for in specialized ACHD centers are better than those presenting in non-specialized units. Diseases like rheumatic heart disease are on the decline, but congenital heart disease will increase as more survivors reach adulthood. In the National University Hospital alone, there are estimated to be 800-1000 ACHD cases above the age of 16 (about half of them on follow-up by paediatric cardiologists). These patients are unlike other cardiac patients in many ways. Their estimated age is 32. They are still studying or training; have careers; are sorting out their lifestyles and having relationships; are getting married and having children; want to live and live to see their children grow and prosper; and contribute to their communities. They need information on career planning, on the wisdom of pregnancy and their contraceptive options, and many other matters. They are often stigmatized at work, and concerned about what they can safely do.They are trying to learn about their conditions and trying to promote their own health. Some patients with good outlooks don’t know they can expect a normal life. Other patients with major health risks don’t know what to watch for, or how and where to access informed care. They are presenting to obstetricians who have no knowledge of how to manage them. They are in emergency rooms with cardiac arrhythmias, heart failure, endocarditis, and chest pain as well as with appendicitis, gallstones, and strokes. PCCS 2006 SPEAKER’S ABSTRACTS November 2, 2006 Prior to 2003 there has not been any setup to care for patients with ACHD in Singapore. In 2002, funding was obtained from the Health Service Development Programme (HSDP), to fund a national programme to care for these patients. This funding was used to train nursing staff, start a cardiopulmonary laboratory and subsidize patient care for device intervention. In 2006, we now have 2 ACHD units in Singapore to care for the needs of these patient. PCCS 2006 S20(2/3) SPEAKER’S ABSTRACTS November 2, 2006 Exercise toterance in GUCH after the surgery Michael A. Gatzoulis, M.D. Royal Brompton Hospital and the National Heart & Lung Institute, London, UK PCCS 2006 S20(3/3) Pregnancy related issues in women with Congenital Heart Disease Most women with mild acyanotic CHD tolerate pregnancy, labor, and delivery well, and their management can be almost same as general population. Patients with moderate to severe CHD require frequent assessment and follow-up by not only obstetricians but also the other specialists such as cardiologists, anesthesiologists, neonatologists and special nurses, who should work as a team. Management consists of limitation of physical activity when appropriate, avoidance of excessive weight gain, use of diuretics to prevent fluid retention, close observation for signs or symptoms of early congestive heart failure with prompt treatment, and control of arrhythmias with antiarrhythmic medications known to be safe for the fetus. Cardiac failure and/or arrhythmia in patients with CHD during pregnancy may be attributable not only to dramatic changes of hemodynamics, hormone, catecholamine and autonomic nervous systems during pregnancy but also underlying anatomy and specific hemodynamics of CHD and scars from prior surgery. Patients with severe cyanotic CHD and reduced functional capacity, Eisenmenger syndrome, or severe cardiac failure constitute a high risk group and should be advised against pregnancy or counseled for therapeutic abortion if pregnancy has occurred. Patients with large aorta, severe left ventricular outflow or inflow stenosis are also high risk for pregnancy and the lesion should be repaired before pregnant if feasible. If surgical correction has been performed without a significant residua or sequelae, the tolerance of pregnancy is nearly normal excluding issue of inheritance. It is essential that the patient should understand the maternal and fetal risks associated with pregnancy. Cardiovasucular surgery or catheter intervention during pregnancy should be considered only in patients who are severely disabled and are not responding to optimal medical therapy. Delivery by cesarean section is indicated only for obstetrical reasons and when there is cardiac instability in spite of medical therapy. Second stage of labor should be as short as possible. The use of prophylactic antibiotics at the time of delivery is indicated in patients with CHD and >moderate risk for infective endocarditis. Hemodynamic and electrocardiographic monitoring should be used in all high-risk patients to identify and correct hemodynamics and electrical instability as soon as possible. Although pregnancy can pose substantial risks for women with CHD, it remains feasible for most with suitable medical support. Pre-pregnancy counseling and multidisciplinary care are essential to help them have their own children at the minimal possible risk and, thus allow them to lead their life potential. SPEAKER’S ABSTRACTS November 2, 2006 Dr. Koichiro Niwa, M.D., FACC Chiba Cardiovascular Center, Chiba, Japan PCCS 2006 S21(1/3) SPEAKER’S ABSTRACTS November 2, 2006 Fetal cardiac pathology Andrew Cook, M.D. Institute of Child Health, University College London, UK PCCS 2006 S21(2/3) Impact of fetal echocardiography: Changing pattern of congenital heart disease SPEAKER’S ABSTRACTS November 2, 2006 In-Sook Park, M.D. Asan Medical Center, Seoul, Korea PCCS 2006 S21(3/3) SPEAKER’S ABSTRACTS November 2, 2006 Progression of CHD During Fetal Life. Yasuki Maeno, M.D. Kurume University School of Medicine, Kurume, Japan Fetal echocardiography provides new viewpoint for congenital heart defects (CHD). Cardiac structures and function of CHD at birth, which we usually observe in neonatal period, are final result of whole process of the heart development during long prenatal period. Fetal echocardiography gives us interesting opportunity to reveal these developmental changes during prenatal period, and several studies have reported this progression of fetal cardiac structure. Although some of these progressions were already speculated from the examination of cardiac structure after birth, some findings were unexpected before prenatal echocardiography. Left and right ventricular outflow tract obstruction makes progressively hypoplastic left and right heart structure, respectively. Prediction for severity of these progressions was reported in several previous studies. However, optimal pre- and perinatal management was still not clear. We still need information to decide the indication of prenatal treatment, and an adequate timing of delivery for these fetuses. Prenatal diagnosis of Transposition of the great arteries (TGA) was proved to improve outcome. Some of the fetuses with TGA were reported to have restrictive foramen ovale, restrictive ductus arteriosus, and developed pulmonary vascular disease. These findings were not even speculated until the prenatal echocardiography found them. Mechanisms of development of theses anatomical changes are still not investigated. Furthermore, appropriate perinatal management for these cases are still unknown. Univenricular heart including heterotaxy syndrome is usually easily detected by prenatal screening. Although several risk factors for the successful Fontan repair had been well investigated for the postnatal cases with these univentricular heart, prediction of progression of these risk factors during fetal period have not been well discussed. For example, atrioventricular valve regurgitation is one important risks for the Fontan type repair, however an amount of this regurgitation at the time of surgery is very difficult to predict during prenatal period. Furthermore, although presence of obstructive pulmonary vein represents poor prognosis, progression of this abnormality after birth is often difficult predict before birth. It is very important to know these pre- and perinatal progressions of the cardiac disease, when we manage these affected fetuses, when we decide the treatment, and especially when we counsel family. PCCS 2006 S22(1/3) Renal replacement therapy SPEAKER’S ABSTRACTS November 2, 2006 Laurance L. Lequier, M.D. Strolley’s Children hospital, Canada PCCS 2006 S22(2/3) SPEAKER’S ABSTRACTS November 2, 2006 LCOS in CICU Zhuoming XU, M.D. Shanghai Children Medical Center, China PCCS 2006 S22(3/3) Expectations and Outcomes for Congenital Heart Surgery Programs Congenital heart disease has moved from a “fatal” disease to a “chronic” disease due to advances in surgical technique and pre- and post- operative care. The treatment approach has progressed from surgeon-centered to multidisciplinary team concept, from Congenital Heart Surgery to Congenital Heart Surgery Programs. As programs mature, the expectations and outcomes of treatments should also progress from focus on mortality to focus on quality/safety and quality of life. Currently, two mortality indices are being tested in many settings, Aristotle and RACHS-1. In the U.S., the Agency for Healthcare Research and Quality is leading the way for quality improvement in healthcare including congenital heart surgery. Recently, Pediatric Quality Indicators have been delineated and efforts are on going to validate them. Finally, quality of life is a more difficult concept to identify and measure especially in the pediatric population. However, treating a “chronic” disease should involve some efforts to monitor quality of life. Examples of such efforts can be seen in the treatment of extremely low birth weight premature newborns, patients with cystic fibrosis, and patients with cancer. SPEAKER’S ABSTRACTS November 2, 2006 Thivakorn Kasemsri, M.D. Medical Director, Pediatric Intensive Care Unit, Covenant Children’s Hospital, Lubbock, Texas, USA PCCS 2006 S23(1/2) SPEAKER’S ABSTRACTS November 2, 2006 PFM coil for VSD occlusion: A real alternative treatment modality Trong-Phi Le, M.D. University of Hamburg, Germany PCCS 2006 S23(2/2) Difficult cases for PDA closure Transcatheter closure of patent ductus arteriosus (PDA) was first performed in 1967 by Dr. Porstman and his associates using Ivalon foam plug. Since then, a variety of devices have been developed to close PDA with variable success. Currently, the most popular device used is Gianturco coil because of easy in delivery, low profile in sheath requirement, high occlusion rate and a low cost. However, high incidence of distal migration of coil was encountered in patients with a large ductus. Coil closure in patients with type B or C ductus also carries a higher percentage of distal embolization. In recent few years, Amplatzer duct occluder has been used to close moderate-to-large sized ductus with a high success rate and complete closure rate. However, deployment of Amplatzer septal occluder is sometimes difficult in young infants with a large ductus. In adult patients with very large ductus where Amplatzer duct occluder may not be sufficient to close the ductus, Amplatzer muscular VSD occluder could be used. Severe pulmonary hypertension is commonly present in adults with a large ductus. Amplatzer duct or muscular VSD occluder could be used for trial occlusion. In contrast to large ductus, small ductus can be closed without difficulty. However, catheter closure of very small ductus is difficult since advancement of catheter across the ductus is difficult. Snare-assisting technique can be useful. In very few cases, guide wire manipulation within the ductus could result in complete occlusion. Therefore, with the advent of devices, transcatheter closure of difficult PDA is feasible and safe in most patients. SPEAKER’S ABSTRACTS November 2, 2006 Jou-Kou Wang, M.D. National University of Taiwan, Taiwan PCCS 2006 S24(1/2) SPEAKER’S ABSTRACTS November 2, 2006 Systemic RV: Natural History Michael A. Gatzoulis, M.D. Royal Brompton Hospital and the National Heart & Lung Institute, London, UK PCCS 2006 S24(2/2) THE MANAGEMENT OF CONGENITALLY CORRECTED TRANSPOSITION OF GREAT ARTERIES This is a complex form of congenital heat disease with many variables needing to be taken into account before arriving at the best management decision for each patient. Repairs that leave the morphological right ventricle (mRV) in the systemic circuit, and a low pressure in the morphological left ventricle (mLV) – physiological repair – have the disadvantage of a significant incidence of mRV failure coupled with progressive tricuspid valve (TV) incompetence. This is similar to the result of Mustard or Senning operation in TGA particularly if a large VSD has been patched. In ccTGA the result is even worse because of the high incidence of large VSD’s, the higher incidence of arrhythmias (particularly complete Heart Block before and after surgery) and the much higher incidence of congenital deformity of the TV. The established results of physiological repair of ccTGA lead to a drive to return the mRV to the low pressure pulmonary circuit (anatomical repair). This was achieved by the double switch procedure consisting of a Mustard or Senning combined with an arterial switch operation, or combined with a Rastelli operation if the pulmonary valve was absent or unsuitable to become an aortic valve. In some patients with failing mRV and TV the mLV has been at low pressure, and mLV retraining with a pulmonary artery band can be considered before anatomical repair. mLV retraining is only reliable at young age while the heart is still growing rapidly. “Anatomical repair”, even in the most favorable patient, cannot be considered a cure, for obvious reasons. It is a long and complex operation. Patients must be carefully selected for suitability, most particularly in the older age group where it may be more prudent to simply replace a TV in the presence of good mRV function, partially relieve LVOTO, apply only a moderately tight PAB, correct a rhythm problem, or transplant, as forms of palliation which are less risky. Certainly, a policy of “Double Switch or bust” is inappropriate. SPEAKER’S ABSTRACTS November 2, 2006 Roger B. B. Mee, M.D. Chairman Department of Pediatric and Congenital Heart Surgery, Cleveland Clinic. (Retired) PCCS 2006 S25(1/2) SPEAKER’S ABSTRACTS November 2, 2006 Pre- and Perinatal Management of Fetal Arrhythmias Yasuki Maeno, M.D. Kurume University School of Medicine, Kurume, Japan Prenatal diagnosis and treatment of fetal arrhythmia is well documented since early 1980’s. Fetal arrhythmia is divided into tachycarrhythmias, bradyarrhythmia, and ectopic beats. Although many prenatal management strategies of fetal arrhythmias have been reported, there are still many issues to be addressed to find the optimal management for each fetus. Fetal tachyarrhythmia consists of supraventricular tachyarrhythmia (SVT) with 1:1 atrioventricular conduction, atrial flutter (AF) with 2:1 atrioventricular conduction, ventricular tachycardia with dissociated atrioventricular conduction, and others. Intrauterine treatment using digoxin is known as a first line treatment in the fetuses with SVT and AF. However, fetuses associated with hydrops or long VA interval SVT are rarely treated by digoxin. Other antiarrhythmic drug such as sotalol, flecainide, or amiodaron, is indicated from the beginning for these fetuses. After birth, some develop to refractory tachyarrhythmia during early neonatal period. Hence close observation after birth is required even after successful prenatal treatment. Fetal bradyarrhythmia consists of atrioventricular block (AVB) with or without structural heart anomaly, and sinus bradycardia. The main cause of AVB without heart anomaly is maternal anti SS-A antibody. Maternal administration of beta-stimulant is effective for increasing fetal heart rate in some of the fetuses. The management of fetuses with maternal SSA antibody is not well established. Since prediction of developing fetal AVB for maternal positive SSA antibody is not established, early detection and early treatment is the currently available perinatal management. Trans-placental steroid administration has been reported to improve the degree of AVB in some of the affected fetuses. Slower fetal ventricular heart rate or presence of mitral valve regurgitation may be early signs to develop fetal hydrops. Dilated cardiomyopathy or endocardial fibroelastosis may be developed during pre- and postnatal period. Further study using large number of patient by multi-center study is required to find optimal pre- and perinatal management for the fetuses with arrhythmias. PCCS 2006 S25(2/2) The Management Implications Following The Diagnosis of Fetal Heart Anomalies The prenatal diagnosis, usually by 20 weeks, of a fetal cardiac abnormality tends to precipitate a crisis for the affected parents. In a setting of grief and emotional distress, there is a challenge to provide meaningful information of the abnormality, the need and risks of any likely interventions and the probable outcomes. That hopefully will enable the parents if allowed the option, to come to an informed decision as to whether to continue with the pregnancy. For any counselling to be credible there must be an accurate and as complete a diagnosis of the cardiac abnormality wherever possible. In addition there is a need for the exclusion of other malformations and/or chromosomal abnormalities. Repeated scans often done to clarify the diagnosis, allows for improved understanding and assimilation of the complex knowledge imparted. Repeated scans are also indicated for a significant arrhythmia where the development of worsening cardiac failure despite medication, may lead to an early delivery once the fetus is viable. Pulmonary compression from increasing cardiac size as for example from severe incompetence of an Ebstein or dysplastic tricuspid valve, may also warrant early delivery. An evolving cardiac lesion such as a hypoplastic left or right heart also necessitates further cardiac scans to help plan management. Throughout the pregnancy there is a need to monitor the well being and growth of the fetus, which if compromised may lead to an early delivery. Any fetus with a major cardiac abnormality or where there is a duct dependent pulmonary or systemic circulation as for example in a severe Fallot’s or critical aortic valve stenosis, warrants delivery at a tertiary centre with appropriate obstetric and neonatal intensive care. A prostaglandin E1 infusion may be required with additional ventilation and/or inotropic support. The early confirmation of the prenatal diagnosis helps plan the immediate neonatal management, and may facilitate mother/infant handling and bonding. Once the diagnosis is confirmed, the anticipated interventions may be proceeded with. The parents previously counselled seem better prepared for the stresses of the surgery that their infant need endure. A prenatal diagnosis also allows for the anticipatory care of the newborn which permits such affected infants to be in as good a state as possible prior to surgical and/or catheter interventions. The fetus with a minor cardiac lesion or one that results in an increasing left to right shunt even if substantial, may be delivered at the parents’/obstetrician’s hospital of choice. Usually the neonate remains asymptomatic until the pulmonary vascular resistance drops SPEAKER’S ABSTRACTS November 2, 2006 Samuel Menahem, M.D. Fetal Cardiac Unit, Monash Medical Centre, Melbourne, Australia SPEAKER’S ABSTRACTS November 2, 2006 PCCS 2006 and the left to right shunt increases. Once again early confirmation of the diagnosis helps reinforce the trust that the parents have in the attending cardiologist who usually has been involved in the prenatal counselling. The diagnosis of a cardiac abnormality in the fetus allows for appropriate counselling of the affected parents. It enables one to plan the management of the pregnancy and perinatal period to ensure an optimal and hopefully improved outcome both for the infant and mother. PCCS 2006 S26(1/3) Risks and mechanisms of sudden death following surgical treatment of congenital heart disease. Michael J. Silka, M.D. Division of Cardiology, Childrens Hospital Los Angeles, University of Southern California, USA SPEAKER’S ABSTRACTS November 3, 2006 Identification of patients at risk for sudden cardiac death (SCD) following surgical treatment of congenital heart disease (CHD) remains a major challenge. In part, this is attributable to the relatively low incidence of unexpected SCD, which has precluded prospective evaluation of the problem. However, this challenge is further complicated by the evolving profile of patients at risk for late SCD, due to the survival of patients with complex defects, along with evolving medical, catheter and surgical treatments for congenital heart defects. In spite of these challenges, population-based studies have provided some insight in to the risks and mechanisms of late SCD in patients with CHD. For patients <20 years of age, the risk of sudden death following CHD surgery is estimated at 1 per 1,000 patient years, compared to 1 per 25,000 patients years in the general population. Three basic mechanisms of late SCD have been identified: arrhythmia, heart failure and thromboembolic events, with approximately 75% of late SCD due to arrhythmias. The types of CHD with long-term survival at highest risk for late SCD are postoperative transposition of the great arteries (atrial switch), tetralogy of Fallot, aortic stenosis and coarctation of the aorta. For some of these defects, the risk of SCD appears to be time-dependent, i.e., increasing incrementally with longer follow-up. Risk stratification for late SCD in adult CHD patients is further complicated by progressive ventricular and AV valve dysfunction, atrial and ventricular arrhythmias related to progressive fibrosis and hypertrophy and possibly late-onset of complete heart block. In addition to the aforementioned defects, SCD has been reported in adults with Ebstein’s anomaly and “corrected transposition” of the great arteries (L-TGA). To date, there have been few reports of late SCD following surgical palliation for univentricular physiology. Given the compromised circulatory physiology and extensive surgical intervention(s), it is anticipated that SCD will become an increasingly prevalent problem in these patients with extended follow-up. In spite of the limited data available, it remains the role of the clinician to assess the risk of late SCD in these patients and make reasonable decisions regarding testing and therapies. This session will conclude with the author’s experience in this field and a proposal for a graded scoring system to assess the risk for late SCD in an individual or group of patients with various forms of CHD. PCCS 2006 S26(2/3) Long QT syndrome SPEAKER’S ABSTRACTS November 3, 2006 Masao Yoshinaga, M.D., Ph.D. National Hospital Organization Kagoshima Cardiovascular & Cancer Center, Japan Long QT syndrome (LQTS) is a rare disease characterized by prolonged ventricular repolarization and a high risk for cardiac events including sudden cardiac death. Most cases of LQTS that come to the attention of physicians are either symptomatic or familial. However, a screening program by ECG conducted for Japanese children (all 1st, 7th and 10th graders) has uncovered large numbers of children with LQTS with no family history and those with no history of LQTS-related cardiac events at the time of diagnosis. 1) Prevalence of LQTS in Japanese children. We measured three consecutive QT and RR intervals of 4,655 children. Their ECGs were recorded when they were 6 years in 1994 and 12 years in 2000. All four children diagnosed as definite LQTS at 12 years did not show characteristic ECG findings of LQTS at 6 years. They are all non-familial cases. The prevalence of LQTS at 12 years old is 1/1,164 (4/4,655). 2) Face immersion test to induce prolongation of QT interval and notched T waves. Recent study has shown that provocation of transient bradycardia followed by sinus tachycardia with adenosine induces abnormal QT prolongation and notched T waves in patients with LQTS. We also showed that face immersion test is a useful bedside test to induce prolongation of the QT interval and T-wave changes in children with LQTS. 3) Correction of the QT interval to minimize the effect of a high heart rate in children. It is well known that Bazett’s formula {(QT)/(RR)k, k=0.5} overcorrects the QT interval in children with a high heart rate. We evaluated the usefulness of 11 formulas in 23,861 1st, 7th, and 10th graders. Exponential or Fridericia’s formulas minimized the effect of the heart rate. The best fit for exponential parameter k for each sex and the graders ranged from 0.30 to 0.35. 4) Risk factors for cardiac events in young patients. A total of 197 Japanese children with LQTS were analyzed. Seven of the 129 (5%) cases without past history and 32 of the 68 (47%) cases with past history of LQTS-related cardiac events experienced new events after diagnosis. Incompliance with medicine and a lower age at diagnosis (p<0.005 for both) were significant predictors for the group with past history. References 1) Fukushige T, Yoshinaga M, et al. Am J Cardiol, 2002 ;89 :395-8. 2) Yoshinaga M, et al. Am J Cardiol, 1999;83:1494-7. 3) Aihoshi S, Yoshinaga M, et al. Jpn Circ J, 1995;59:190-7. 4) Yoshinaga M, et al. Circ J, 2003 ;67 :1007-12. PCCS 2006 S26(3/3) Patients with Right Atrial Isomerism Mei-Hwan Wu, M.D., Ph.D. Departments of Pediatrics, National Taiwan University, Taipei, Taiwan SPEAKER’S ABSTRACTS November 3, 2006 Right atrial isomerism is nearly always associated with complex congenital heart disease, typically a combination of anomalous pulmonary venous return, common atrium, common atrioventricular valve, dominant right ventricle, double outlet right ventricle and pulmonary stenosis (atresia). Most of the patients need a palliative shunt with or without rerouting of the obstructed pulmonary veins, and later staged total cavopulmonary connection. The prognosis has been poor with an estimated 5-year survival ranged from 29 to 51 %. Moreover, probably due to the residual hemodynamics abnormalities, abnormal cardiac conduction system, asplenism and gastrointestinal anomaly, these patients are also at risk of sudden death even palliation. The sudden death rate may range from 5% to 14 %. From 1980 to 2005, 257 patients (1,171 patient-years) were diagnosed as RAI in this institution. The one-year, 5-year and 10 year survival was 66%, 49% and 41%, respectively. There were a total of 29 sudden deaths (acute cardiovascular collapse from which biological death occurred within 24 hours) (24 events/1,000 patient-years). Sudden death tended to occur in infancy or early childhood. The freedom of sudden death was 85 % at the age of 3 years and remains relatively stationary thereafter. The mechanisms were classified as sudden tachyarrhythmic in 2 (6.9%), sudden cardiac but nontachyarrrhythmic (sudden onset severe cyanosis) in 17 (58.6%), and sudden noncardiac in 10 (34.5 %) (fulminant sepsis with positive blood culture: streptococcus pneumonia 6, E. coli 1 and yeast-like organism 1). The age of the life threatening infection ranged from 5 months to 4.5 years (median 17 months, 18 15 months). Among the 257 patients, we found that the presence of obstructed non-cardiac drainage of pulmonary venous return (42%), pulmonary atresia/ stenosis (31/59%), major aortopulmonary collateral arteries (5%), aortic arch coarctation or interruption (2.3%) and hital hernia (16%), or the occurrence of narrow QRS tachycardia (24 %) was not associated with a higher risk of sudden death. It is concluded that the sudden death in patients with right isomerism may be related to the complex cardiac anomalies per se, a susceptibility to fulminant peumococcal infection, and only rarely arrhythmia. Early vaccination against the pneumococcus may reduce the risk of fulminant infection. PCCS 2006 S27(1/3) HAEMODYNAMIC MONITORING: TOOL OR TOY? SPEAKER’S ABSTRACTS November 3, 2006 Dan Penny, M.D., Ph.D., FRACP Director of Cardiology, The Royal Children’s Hospital Professorial Fellow Dept. of Paediatrics, University of Melbourne, Australia In 1929, a German surgeon, Werner Forsmann, introduced a catheter into his right atrium from an arm vein. This was the first time that a mixed venous saturation was measured in a human, but the technique was considered unsafe for routine application in humans. In 1941, Andre Cournand, who is considered to be the pioneer of cardiac catheterisation, published his paper on “catheterisation of the right auricle in man”. He had perfected the technique for safe catheterisation of the right heart, in health and disease. This report represented a landmark for cardiologists and cardiovascular physiologists, for two important reasons. First, this opened a ‘Pandora’s box’ in the field of cardiovascular monitoring and investigation. Second, it enabled the practical application of the Direct Fick measurement of cardiac output for the first time, since it was described by Adolph Fick in nearly 70 years previously. The thermal indicator technique (thermodilution) of measuring cardiac output was introduced by Fegler in 1954, and this was further refined, with the advent of the Swan Ganz catheter in 1971. Over the past 3 decades, there has been huge literature on the pros and cons of cardiac output measurement in intensive care. The technique enjoyed periods of near-routine use in adult intensive care practice, but more recently has been used less frequently. This is because the evidence base in favour of so-called ‘goal-directed’ therapy is limited, and there are some safety concerns associated with pulmonary artery catheterisation. Invasive monitoring in children in intensive care, has – as for many techniques in paediatrics – lagged behind adult practice. It is really only in post-operative cardiac intensive care, that the use of haemodynamic monitoring has extended beyond the arterial, or central venous line. Nowadays, a major paradox exists in post-operative cardiac intensive care. As our patients become increasingly complex, we are moving away from multiple, invasive monitoring. For example, pulmonary artery pressure monitoring catheters were previously ‘routine’ in many neonates undergoing surgery, and in infants with large septal defects. These catheters are now rarely used, due to concerns regarding catheter-associated thrombus, infection, embolus, or tamponade upon their removal which must be balanced against the benefits associated with their use. The same risk can be applied to cardiac output monitoring, whether using the traditional pulmonary artery thermodilution technique, or the more recent transpulmonary thermodilution (PICCO) technique. PICCO has recently been introduced into paediatric practice, and requires the placement of a small femoral artery catheter. PCCS 2006 SPEAKER’S ABSTRACTS November 3, 2006 Cannulation of the femoral artery is generally avoided in paediatrics outside of the catheter laboratory, due to significant risks of thrombosis, and theoretical risks associated with limb growth, and so it is unlikely that the use of PICCO will be widespread in cardiac intensive care. Cardiac output monitoring in paediatric cardiac intensive care has rarely been used outside of the research setting, and there is little, if any, evidence to support its routine application. Indeed, we still do not know what is a ‘normal’ cardiac output for an infant after an arterial switch operation, or a larger child after a Fontan operation. Furthermore, in infants with a functionally univentricular heart, the measurement of total cardiac output is relatively meaningless, given that we are largely focussed on systemic oxygen delivery. In the absence of compelling supporting evidence, it is unlikely that the invasive measurement of cardiac output will become more routine in cardiac intensive care. In the modern era, morbidity and mortality early after cardiac surgery is almost exclusively confined to the neonate and young infant with complex heart disease. In particular, with the functionally univentricular circulation, after Norwood-type operations. In these infants, systemic oxygen delivery is the parameter which is of greatest relevance, and paradoxically the most difficult to measure accurately. As clinicians caring for these patients, most of us would welcome additional haemodynamic monitoring tools to ‘guide’ us, or reassure us that our patients are doing well. Conversely, additional information which will herald haemodynamic deterioration at an early stage, would be very desirable in this fragile population. There is some limited literature that continuous monitoring of central venous oxygen saturation, can be used as a guide to the adequacy of systemic oxygen delivery. This technique requires the placement of a catheter directly into the superior caval vein, which can be used for sampling of mixed venous blood. As we have already discussed, this technique is not without its own inherent risks, and the search for a ‘surrogate surrogate(!)’ has resulted in the increasing use of non-invasive cerebral oxygen saturation monitoring in the Norwood population. This technique is appealing in that it is non-invasive and has no known direct risks, but requires further validation in the neonatal cardiac population. A number of other haemodynamic monitoring tools are coming into vogue in clinical intensive care. These include transthoracic or oesophageal Doppler to monitor stroke volume, which has recently been introduced into paediatric (non-cardiac) practice. Tools which target the assessment of regional oxygen delivery are also becoming available in adults: these include spectral monitoring of the tissue microcirculation (in sepsis), regional perfusion, and tissue oxygen monitoring. The usefulness of these in paediatric cardiac intensive care is currently unknown, and their will likely be a typical time-lag between their application in adults and children. Ultimately, many of these techniques can be regarded as ‘toys’, or perhaps less harshly could be considered as research tools in paediatric cardiac intensive care. Right now, we do not yet have an accurate, reliable, and risk-free measure of systemic oxygen delivery. Until we do, we will continue to rely on ‘surrogate’ markers of perfusion such as acid-base balance, lactate, mixed venous oxygen saturation, and end-organ function. PCCS 2006 S27(2/3) Cardiopulmonary interaction: How principles become practices? SPEAKER’S ABSTRACTS November 3, 2006 Satid Thammasitboon, M.D. West Verginia University School of Medicine, USA PCCS 2006 S27(3/3) Nutritional support and immunocompetence Amit Varma, M.D. Fortis Health Care, New Delhi, India SPEAKER’S ABSTRACTS November 3, 2006 PCCS 2006 P3(1/2) Pathologist’s bench: A New Look at Cardiac Development SPEAKER’S ABSTRACTS November 3, 2006 Andrew Cook, M.D. Institute of Child Health, University College London, UK PCCS 2006 P3(2/2) Cardiologist’s aspect of genetics of congenital CV disease: From molecular bench to bedside Makoto Nakazawa, M.D. Sounthern TOHOKU General Hospital, Sounthern TOHOKU Research Institute, Fukushima, Japan SPEAKER’S ABSTRACTS November 3, 2006 PCCS 2006 S28(1/3) Paediatric Cardiac Services in Pakistan SPEAKER’S ABSTRACTS November 3, 2006 Kalim Uddin Aziz, M.D. Professor (Emiratus) of paediatric Cardiology National Institute of Cardiovascular Diseases, Pakistan The first exclusive Paediatric Cardiology Services was started at National Institute of Cardiovascular Disease at Karachi in 1981. Invasive or noninvasive services were instituted which involved out patient service for children under 12 years age, echocardiography. Mmode in the early days and then 2-D Color Doppler in 1983. Cardiac Catheterization for all age groups was started in 1981. The cadiac catheterization service was mostly diagnostic except for balloon atrial septosmy . Balloon dilatation were started in 1987 and later device deployment for congenital lesions was started beginning with Coil Closure of PDA at Armed Forces Institute Rawalpindi and in Punjab Institute of cardiology at Lahore. At present catheter intervention services are available in two institutes in Karachi, two at Lahore and one in Rawalpindi. Integrated training program of training of Catheter Therapeutics, by involving consultants form England, America, was started in1994 and the end result is that catheter intervention is now a major component of paediatric catheterization work around the country. Fellowship training program for Paediatric Cardiology culminating in FCPS by the College of Physicians and surgeons Pakistan, was initiated at NICVD in 1991, culminating in FCPS in paed cardiology, by the college of Physician and Surgeons of Pakistan (similar to board certificate).The fellowship program is available in two institutions, AKU and NICVD at Karachi, two Punjab Institute of Cardiology and Children Hospital, in Lahore and one Armed Forces Institute, at Rawalpindi. While surgery in older children is being performed in five hospitals started since 1981. The man power and infrastructures for surgical services for infants are being developed. Three centers, one in Karachi (2006), one in Lahore (2000) and one in Rawalpindi (2001) have dedicated Paediatric Cardiology surgeon and who are performing corrective surgery in the infants.. In conclusion development of Paediatric Cardiology services are progressing at a rapid rate in Pakistan and the goal is to correct congenital cardiac malformation at an early age PCCS 2006 S28(2/3) CONGENITAL HEART DISEASEMANAGEMENT IN DEVELOPING COUNTRY: THE INDONESIAN EXPERIENCE Anna Ulfah Rahajoe, M.D. President, National Cardiovascular Center, Jakarta – Indonesia SPEAKER’S ABSTRACTS November 3, 2006 At a crude birth rate of 20.34/1000 in Indonesia, the total annual live-births would be approximately 5 million. Considering the estimate of 9/1000 live-births, approximately 45.000 infants with CHD are added every year to the total pool. About 45% of CHD cases require some form of intervention or surgery during childhood. Thus, every year, approximately 20.000 children require surgery or care at an advanced infant cardiac center. The number of surgery and intervention for CHD in the whole country is less than 1000 cases per-year. Majority CHD cases are undetected, leading to high infant mortality rate of 34.39 deaths/1,000 live births. The development of non surgical intervention has led to a significant increase in the number of conditions which are now amenable to treatment in the cardiac catheter laboratory. Balloon Pulmonal Valvuloplasty is the treatment of choice for pulmonal stenosis, as well as Balloon Atrial Septostomy in some types of cyanotic CHD. Closure of the patent arterial duct, atrial and ventricular septal defect using Amplatzer devices tend to increase every year. While this approach has considerable benefits over surgery, in Indonesia the cost is higher than surgery. In two years periode (2003-2005), 1336 patients had open and closed heart surgery at the National Cardiovascular Center, the mortality rate was 5.56%. Almost 70% were ductus ligation, atrial or ventricular septal defect (ASD/VSD) closure, and tetralogy of Fallot repair. Surgery in infancy tend to increase, during this periode there were approximately 30% of the total case. Arterial switch operation, repair of atrioventricular septal defect and coarctation of the aorta associated with other lesions usually performed in neonates or infancy, the mortality rate in this particular cases is still high (15-20%). Evaluation on VSD cases underwent surgery in 2000 – 2004, confirmed the high incidence of subarterial doubly committed VSD, 26.6% and 43.6% in childhood and adult respectively; while muscular VSD were less than 2%. The resources are not only limited but also are at times improperly utilized. The existing number of trained personnel for pediatric cardiology and pediatric cardiac surgery is inadequate. Above all there is no national policy for pediatric heart care. In conclusion, pediatric cardiac care in Indonesia is still in its infancy. The barriers are: lack of awareness on CHD, limited resources and facilities, high cost of treatment and limited financial support. Training programs exclusively dedicated to pediatric cardiology and pediatric cardiac surgery need to be established in centers with good standards of pediatric cardiac care. Keywords : Congenital heart diseases; Pediatric cardiology; Pediatric cardiac surgery; Pediatric cardiac care PCCS 2006 S28(3/3) ‘Heart to Heart’ Project for Vietnamese Children - Joint Heart Project between SMC & NHP - SPEAKER’S ABSTRACTS November 3, 2006 Heung Jae Lee, M.D. Professor of Pediatrics, Sungkyunkwan University School of Medicine Samsung Medical Center, Seoul, Korea Korean Experience during the Past 5 Decades: Korea’s infrastructure including economic, social, medical and educational systems collapsed after the Korean war, and rebuilding efforts started from mid- 50s. Open-heart surgery was first introduced in Korea in 1959, thanks to the Minnesota Plan, a program sponsored by the United Nations to rebuild higher education in Korea. But we had to wait for another 20 years to see congenital heart surgery become popular. During these years, the majority of pediatric heart diseases seen in Korean hospitals were kids suffering from rheumatic heart disease, diphtheric myocarditis, pyogenic and tuberculous pericarditis. Only some children suffering from congenital heart disease could be saved after heart surgery in the US, thanks to the help of the International Human Assistance Program, a US charity program. Unfortunately more young lives, however, perished without proper treatment. The national social security system was established in 1977, which provided momentum for promoting heart surgery for children. This year, Korea’s gross national product (GNP) reached 944 US dollars. And it was this year that one cardiac center started to performed over 100 open-heart surgeries per year, for the first time in Korea. In the early 1980s, Korea’s GNP surpassed 1,000 US dollars and various circles from the society participated in the campaign to help children with heart disease. And this campaign played a decisive role in the rapid development of heart surgery in Korea —the number of heart surgical operations increased and the medical technology improved. Also, the Korean Heart Foundation was set up and offered monetary assistance every year to 1,000 children suffering from congenital heart disease for their operations. In addition, religious organizations and charities led the campaign for charity operation. As a result, every single young patient from a poor family was able to receive heart surgery without concerns about medical fees since the mid-1980s. Technological improvement was also remarkable. Surgical technology for newborn infants with complex CHD developed fast. With these achievements, Korea became almost equal to advanced countries such as the US and European countries in surgical technology after the mid-1990s. For instance, in Samsung Medical Center, one of the 5 major cardiac centers in Seoul, there has been no single mortality with the arterial switch operation, even for the complex transposition patients, during the past 10 years. PCCS 2006 SPEAKER’S ABSTRACTS November 3, 2006 With the society maturing, Korea has offered charity operations for young patients with congenital heart disease in neighboring developing countries in Asia, since the early 1990s, when the Korean GNPs surpassed 5,000 US$. Also, surgical training for doctors in these countries and medical technology transfer has been provided. As mentioned, Korea overcame post-war hardship very successfully. The two major factors for this success were: 1) Rapid economic development; 2) Overseas training for medical experts and their commitment. ‘Heart to Heart’ Project for Vietnamese Children: ‘Heart to Heart’ is a 5-year joint heart project between National Hospital of Pediatrics (NHP) in Hanoi and Samsung Medical Center (SMC) in Seoul, for the Vietnamese children with congenital heart disease, with the financial support from Samsung VINA Electronic Company. The project started in September, 2003, when the ‘NHP-SMC joint heart surgical team performed, for the first time in NHP, open heart surgeries on Vietnamese kids with congenital heart disease. From that time on, the ‘NHP-SMC Joint Heart Project’ has been carried out in two directions as follows: First, technical support from SMC has been provided in order for the pediatric heart surgical team to be established at NHP. To this end, medical staffs from both hospitals have visited each other from time to time. During the past 3 years, the SMC congenital heart team from Seoul has visited Hanoi 6 times to cooperate with NHP, and the 7 groups of 26 medical staffs from NHP has visited SMC to attend short term (1~3 months) training course. Long term training programs will be offered to young and competent medical staffs during the later segment of the project. Second, charity medical treatment and free surgery for the Vietnamese children with congenital heart disease is one of the major on-going programs during the period of five year project. The surgery has been jointly conducted by medical staff from both hospitals at either NHP or SMC, depending on the situation. During the last 3 years, 25 joint open heart surgery between both hospitals and 2 joint interventions has been performed at NHP, and 8 other complicated patients has been transferred to SMC for surgery, together with the NHP staffs. Since 2005, when the surgical team at NHP become reasonably well-established, the team has take the lead in providing this treatment service for the kids with relatively simple congenital heart disease. So far, pediatric hear surgical team in NHP has performed over 250 open heart surgery by themselves, and the catheter interventions are regularly carried out by the NHP staffs. ‘Heart to Heart’ Project is a comprehensive and integrated joint project between two hospitals, one in developed and one in developing countries, in the field of congenital heart disease, which includes collaborations in high tech treatment services and education programs, and also in charity activities. The project will evolve into collaboration in academic and research activities as long as it is possible. PCCS 2006 S28(3/3) Heart to heart project: Between National Hospital of Pediatrics (Vietnam) and Samsung Medical Center (South Korea) SPEAKER’S ABSTRACTS November 3, 2006 Nguyen Thanh Cong, M.D. 9 Cao Ba Quat, Badinh, Hanoi, Vietnam National Hospital of Pediatrics is located in Hanoi, Vietnam, with 500 beds. It is a highest level of pediatrics in the North of Vietnam. Situation of cardiology in National Hospital of Pediatrics before project (2002): Department of Cardiology has 30 beds, 6 cardiac pediatricians, Diagnoses are based on only echocardiography. In Surgical Department, with 4 cardiothoracic surgeons the possible surgical treatments are: PDA and palliative procedure for Tetralogy of Fallot. The aims of Project: Helping N.H.P. to develop pediatric cardiology and pediatric cardiac surgery. The project has got the financial support from Samsung Electronics Company. Every year, there are the teaching programs in Samsung Medical Center and practical training courses in N.H.P. Three years after the Heart to heart project, a big step has been done. One open heart surgery team and one cardiac intervention team have been organized and are capable for independent every day activity. In Department of Cardiology, doctors could give more precise diagnoses, 400 cardiac interventions have been done. For open heart surgery, one operating theatre with one cardiopulmonary bypass pump from Terumo Company and 2-beds CICU was also established, 2 doctor of CICU have got training course in S.M.C. Five first cases of open heart surgery (4 ASD, 1 VSD) were operated on successfully during second week of Sep. 2003 with the help of cardiac surgery team from Samsung Medical Center. Up to now, 300 cases of open heart surgery has been done, there are ASD, VSD, AVSD, TAPVC, and Tetralogy of Fallot. The lowest body weight was 4.5 kg. With the success of first step, N.H.P and S.M.C. would like to share their experiences with other children cardiac service in developing countries and hope that N.H.P. could become the highest-level center of pediatric cardiology for the North of Vietnam in 5 – 10 years period. PCCS 2006 S29(1/2) Acyanotic CHD Ms. Lily Lihwa Hsu, R.N., MSN. Ms. Li-Juan Fu, R.N. Shanghai Children Medical Center, China SPEAKER’S ABSTRACTS November 3, 2006 PCCS 2006 S29(2/2) Cyanotic CHD SPEAKER’S ABSTRACTS November 3, 2006 Bernadette McCormick, BSN, RN Rady Children’s Hospital, San Diego, CA USA Designed for Nurses working with Children with Congenital Heart Defects (CHD), this presentation will include a review of common cyanotic CHD’s anatomy and physiology, and common treatment strategies. PCCS 2006 LS3(1/3) Introduction Transcatheter closure of perimembranous and subpulmonary VSD : immediate term follow-up for late AV block Background: Ventricular septal defect (VSD) is the most common congenital heart disease. Traditional surgical correction is the procedure of choice for closure of VSD. Transcatheter device closure using AmplatzerTM Perimembranous VSD device has emerged as an alternative treatment. Objectives: To report an immediate results with follow-up for patient underwent transcatheter closure of VSD. All cardiac rhythm pre and post procedure were recorded up to 6 months. Materials and methods: We collected data from patients who had transcatheter closure of VSD from February 2004 to June 2006. A peri-procedure ECG were reviewed in each patients for abnormalities such as right bundle branch block (RBBB) or complete 3rd degree atrioventricular block (3AVB) and follow-up ECG was done within 1 year post procedure. Results: 39 patients were included in this study. Their age varied from 1 to 34 years old (median age = 13.3 10.7 yrs). The average body weight was 38 25 kg. There were 34 pre-procedure ECG available for reviewed. 27 of them had normal ECG with 6 of them with RBBB and 1 of 1AVB with RBBB. There was successful transcatheter closure of VSD in 38 out of 39 patients. The diameter of VSD varied from 4.5 to 14mm (mean VSD diameter = 7.6mm.). The average device Size: VSD size (DV ratio) was 1.2 0.19. One patient did not have VSD device closure due to transient AVB. Type of VSD closed were Perimembranous VSD (n=28), subpulmonary infundibular type(n= 8), midmuscular (n =2) and right ventricular systolic pressure (RVSP) was 38?12 mmHg. Immediate post deploys 6 more patients developed RBBB post-procedure. Two of them had 3AVB out of hospital during the 1st week post-closure. One patient recovered after steroid treatment within one week (DV=1.33). The other had persistent 3AVB with symptom and had permanent pacemaker placed two months later (DV=1.4). Late 3AVB was noted in one asymptomatic girl (2 years old, DV=1.6). All three patients had perimembranous VSD. Conclusion : Transcatheter closure of VSD appears effective and relatively safe procedure. AVB was noted in 7.8% of patients. One permanent pacemaker was placed. Although there was no clear risk factors identifies. It appeared that the appearing of RBBB postprocedure may have some relationship with post-procedure early or late 3AVB. SPEAKER’S ABSTRACTS November 3, 2006 Kritvikrom Durongpisitkul, M.D. Division of Cardiology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand PCCS 2006 LS3(2/3) What does the Interventionist need to know about the anatomy of VSD? SPEAKER’S ABSTRACTS November 3, 2006 James L Wilkinson, M.D. Honorary Cardiologist, Royal Melbourne Hospital, Melbourne, Australia The anatomy of the ventricular septum is complex. A clear understanding of the normal anatomy, as well as a knowledge of the sites of septal defects and their relationships to adjacent structures, is important. The anatomy of the membranous septum, the conducting system and the semi-lunar valves and awareness of the implications of septal defects in this area is vital. The suitability of different types of VSD for device closure depends on their location and proximity to these and other structures within the heart, as well as to the size of the septal defect. This presentation will cover important features of normal anatomy as well as the issues presented by septal defects at various locations within the ventricular septum. PCCS 2006 LS3(3/3) Transcatheter Closure of Ventricular Septal Defects: Results & Complications Ziyad M. Hijazi, M.D., MPH, FSCAI The University of Chicago Hospitals and The Pritzker School of Medicine, USA SPEAKER’S ABSTRACTS November 3, 2006 Ventricular septal defects (VSD) are the most common congenital cardiac anomalies, accounting in isolation for almost 20% of congenital cardiac lesions. Ventricular septal defects have also been reported after myocardial infarction, trauma as well as residual defects after prior attempts at surgical closure. Depending on size, VSD’s can cause variable degrees of left-to-right shunting with or without pulmonary hypertension. Small muscular defects diagnosed in infancy have a high likelihood of spontaneous closure, whereas larger defects tend to persist through adulthood. The likelihood of spontaneous closure decreases with advancing age and is less common for defects located within the perimembranous region of the ventricular septum. Irrespective of the hemodynamic effects of VSDs there is an additional risk of developing subacute bacterial endocarditis. Surgical closure has been advocated as the gold standard for treatment of large VSD’s. Surgical closure of perimembranous VSD’s yields excellent results with low morbidity and mortality. However, the outcome is less favorable in patients with increased surgical risk factors, multiple previous cardiac surgical interventions, poorly accessible muscular VSDs or “Swiss-cheese” type VSDs. In 1988 Lock and colleagues reported the first experience of transcatheter closure of muscular VSD’s in 7 patients using the Rashkind double umbrella device. Since then different devices have been used to close muscular as well as perimembranous VSD’s with variable success, such as the Clamshell / CardioSeal device, the buttoned device as well as detachable coils . Since 1998 different types of Amplatzer devices have been used to close VSD’s . Devices for closure of VSD’s have not only been used in the cardiac Catheterization laboratory. In 1993 Fishberger and colleagues first reported intraoperative VSD device closure using the Rashkind double umbrella device in 10 patients. More recently Bacha and colleagues reported perventricular device closure of muscular VSDs without cardiopulmonary bypass, using the Amplatzer mVSD device in 6 patients. In this presentation, I will discuss device closure of muscular and PmVSD using the Amplatzer devices and outline their results including the complications encountered during and after the closure. PCCS 2006 S30(1/3) Transcatheter Closure of ASD: Tips and Technique SPEAKER’S ABSTRACTS November 3, 2006 Dr. Geoffrey K Lane, B. Pharm, MB.BS, FRACP Director Cardiac Catheterisation and Interventional Cardiology Royal Children’s Hospital Melbourne, Australia Success in percutaneously closing atrial septal defects with any device is dependent upon proper case selection. In general, patients with “deficiency” of more than 2 margins of the fossa ovalis should be considered unsuitable for percutaneous closure. Assessment of atrial septal defect suitability is often performed with transthoracic echocardiography and refined with either trans-oesophageal or intracardiac echocardiography at the time of catheterisation. The use of balloon sizing remains the “gold standard” for device selection. The most frequently used method is static balloon sizing utilising the “stop flow” end point. The choice of device depends largely on the size of the defect to be closed. For small to medium sized atrial septal defects, either the Amplatzer Septal Occluder or the Helex Septal Occluder are equally effective. Device sizing varies depending on which device is chosen for implantation. For Amplatzer Septal Occluder implantation, a device size equal to the stretched balloon size or no greater than 1mm larger should be considered. For Helex Septal Occluder implantation, the device should be at least 1.7 times larger than the stretched balloon size. Defects with a stretched size of greater than 18mm should be considered unsuitable for use with a Helex Septal Occluder. For closure of large atrial septal defects, the use of additional techniques or speciality delivery sheaths may need to be utilised. PCCS 2006 S30(2/3) Transcatheter closure of VSD: Asian perspective Mazeni Alwi, M.D. National Heart Institute, Kuala Lumpur, Malaysia SPEAKER’S ABSTRACTS November 3, 2006 PCCS 2006 S30(3/3) Technique of balloon dilation of aortic coarctation SPEAKER’S ABSTRACTS November 3, 2006 Mohammed Omar Galal, M.D., Ph.D., MBA King Faisal Specialist Hospital & RC, Jeddah, Saudi Arabia An interventional catheterization procedure is like every other process; a series of clearly defined steps that achieves a projected end result. As a matter of fact procedures are done in the same way each time. They almost follow a structured format. Balloon dilation of aortic coarctation is no exception. As goes for many other interventions, the process of balloon dilation will first follow a linear type of procedure, where primarily basic tasks that contain sequential steps are needed. The conditions and basic tools of the first linear part is a functional cardiac catheterization laboratory, excellent technical staff, availability of the materials and tools needed for access, establishing diagnosis- in our example an excellent aortic angiography to visualize the coarcted segment. In other words, this part includes the preparation for the actual balloon dilation. This is then followed by a decision or branched type procedure. The decision has to be made: is this specific case for balloon dilation or not, if yes, which balloon to aortic diameter ratio to use, which balloon manufacturer is best for this case etc. When the decision is taken, a linear type procedure is followed to proceed with the actual intervention and than conclude the examination. While the beginning and the last part are more or less the technical and hence easy learnable, the main part is the clinical part and needs besides knowledge, also experience and intuition to make the right decision for the specific patient and lesion. In the context of our presentation, the different steps of this procedure will be addressed and highlighted, the potential risks and complications will be also mentioned. PCCS 2006 S31(1/3) Conduction system in CHD Andrew Cook, M.D. Institute of Child Health, University College London, UK SPEAKER’S ABSTRACTS November 3, 2006 PCCS 2006 S31(2/3) Supraventricular tachycardia and catheter ablation in complex biventricular forms of congenital heart disease. SPEAKER’S ABSTRACTS November 3, 2006 Michael J. Silka, M.D. Division of Cardiology, Childrens Hospital Los Angeles, University of Southern California, USA Catheter ablation has emerged as a highly effective and low risk definitive therapy for patients with supraventricular tachycardia (SVT). However, the success rates for catheter ablation have remained significantly lower for patients with congenital heart disease, along with a greater risk of procedural complication, most notably advanced AV block. In this session, the mechanisms of SVT, abnormalities of conduction system anatomy and approaches to catheter ablation for patients with complex bi-ventricular congenital heart defects will be discussed. This include patients with AV septal defects, Ebstein’s anomaly, discordant AV connections (L-TGA) and patients with d-transposition of the great arteries (d-TGA) following an atrial “switch” procedure. In any of these defects, the presence of intrinsic conduction system abnormalities such as bundle branch block or conduction system displacement may render ECG prediction of accessory AV connection location inaccurate. Furthermore, AV node re-entrant tachycardia may often occur as a second mechanism of SVT in these patients. For patients with an AV septal defect, awareness of the posterior (inferior) displacement of the conduction system is paramount. Compared to the normal location of the AV node-His bundle, the conduction system is displaced in an AVSD to the posterior aspect of the triscuspid valve annulus. Ablation of accessory AV connections in the posterior or posterior-septal region may result in simultaneous injury to the normal conduction system. The anatomic substrate of Ebstein’s anomaly presents several unique challenges in the performance of catheter ablation. These include accurate definition of the anatomic AV groove (site of accessory pathway insertion), the presence of multiple accessory AV connections along with the fractionation of electrograms which complicates the interpretation of intracardiac signals. For patients with marked right heart dilation, catheter positioning and stability also remain formidable technical challenges. Although accessory AV connections are commonly reported in association with discordant AV connections (L-TGA), minimal data exists regarding the ablation of these pathways. It is important to appreciate the anterior displacement of the AV conduction system in this defect compared to normal, and to limit catheter manipulation in this region. The majority of accessory AV connections in L-TGA occur in the posterior septal region. However, due to anatomic distortion and displacement, cannulation of the coronary sinus may be difficult in these patients. PCCS 2006 Following an atrial switch procedure (Mustard or Senning), intra-atrial re-entrant tachycardia is the most common mechanism of SVT. For some patients, RF ablation along the cavo-tricuspid isthmus provides cure for this arrhythmia. However, in others, the arrhythmia is incision related and may require more complex mapping techniques. Caution with attempted ablation in the posterior-septal space is required, as AV block has been reported during attempted ablation in this region. SPEAKER’S ABSTRACTS November 3, 2006 PCCS 2006 S31(3/3) SVT and complex single ventricle and post Fontan operation SPEAKER’S ABSTRACTS November 3, 2006 Eun Jung Bae, M.D., Ph.D. Associate professor Department of Pediatrics, Seoul National University College of Medicine, Korea Because of recent advance of surgical results, many children with complex congenital heart disease have survived after corrective or palliative repair. However, tachyarrhythmia is recognized to be an important source of early and late morbidity and mortality especially in post-Fontan patients. Intraatrial reentrant tachycardia (IART) and atrial fibrillation have been known to be major tachycardias following Fontan type palliation. Various treatment modalities such as antiarrhythmic medications, pacemaker implantation, transcatheter ablation, and Fontan conversion with arrhythmia surgery have been tried successfully. Recently we reported the coexistence of two distinct AV nodes in complex congenital heart disease and its incidence and relation to arrhythmias. From 2001 to 2003, we performed an electrophysiologic study upon 52 consecutive patients who had undergone cardiac catheterization after Fontan completion. Atrial pacing was performed at 3 or more different atrial sites. In 10/52 patients, two different QRS complexes were recorded at different pacing sites, suggesting twin AV node (9/20 in right atrial isomerism, 1/8 AV discordance, 0/24 other complex anomalies). AV reciprocating tachycardia (AVRT), presumably involving two AV nodes and a connecting sling, was induced in 6 of 10 patients who had twin AV node (4/6 used posterior AV node as an antegrade limb, 2/6 used an anterior AV node as an antegrade limb). Heterotaxy syndrome (p<0.001) and complete atrioventricular septal defect (p=0.002) were found to be risk factors for twin AV node. Junctional tachycardia (JT; HR>150/min) with either VA dissociation (7/9) or second degree VA block (2/9) were induced by pacing or isoproterenol infusion in 9/52 patients. Junctional tachycardia induction was associated with a twin AV node (p=0.04), or a history of early postoperative junctional ectopic tachycardia (p=0.02). A complicated AV node conduction system such as twin AV node was frequent in heterotaxy syndrome. Both AVRT, and JT with VA block may be important causes of tachyarrhythmia in this patient group. PCCS 2006 S32(1/2) Patient and Family Education. Bernadette McCormick, BSN, R.N. Rady Children’s Hospital, San Diego, CA. USA SPEAKER’S ABSTRACTS November 3, 2006 This presentation will review the current data on long-term physical and developmental outcomes of children with Congenital Heart Defects. The focus will be on patients with complex lesions. PCCS 2006 S32(2/2) Supporting Family with Congenital Heart Disease Child SPEAKER’S ABSTRACTS November 3, 2006 Lily Lihwa Hsu, RN., MSN Program Director Nursing Education-Shanghai Deputy Dean Shanghai Jiao Tong University School of Nursing, Shanghai, China Family with Congenital Heart Disease (CHD) child frequently suffers from stress, uncertainty, and guilt. Stress may come from disease treatment and complication that occur during surgery and recovery period. Feeling of uncertainty is frequently associated with long-term unpredictable outcome or cardiac function after corrective or palliative surgery. Many families with critically ill child staying in critical care setting also have unmet needs that are important to be recogni Family with critical ill child has many needs, that including to be with and be able to participate in child care, to receive accurate consistent information on the case, to discuss in depth with physicians, to feel that everything possible is done for their child, to have an appropriate place to stay overnight near ICU, to feel respected by health professionals while taking care of their child, to feel hopeful about the child’s survival outcome, and to know the child is free from pain. In addition, it is essential to assess family condition during critical time. The assessment should include family’s level of anxiety from ICU setting, family’s coping style, their concerns on worry and pain, loss of parents normal caregiver’s role, loss of control while being hospitalized, feeling of inadequacy, restricted visiting rules, and the appearance of the ill child. Also, the condition of other patients nearby may worsen the stressful condition. All of the above conditions require health care professionals to provide extra support to these families to help them to overcome their uncertainty or fear while their child is in critical care situation. In order to serve parents of child with Congenital Heart Disease, the family-centered approach was initiated in Shanghai Children’s Medical Center (SCMC) cardiovascular surgery units. A descriptive study was conducted in 6 units to identify the gap between what parents perceive care should be, and how they view nurses’ actual practice in the family-centered care. A total of 180 parents were selected by purposive sampling. The study result indicated that nurses were able to meet the physical needs of hospitalized children and their parents, able to assist parents for them to be closer to their child, and willing to help children to feel “well cared for”. However, data also indicated that nurses have gap to respond to children’s behavioral manifestation, psychological needs, and emotional reaction related to illness or hospitalization. The above study suggests that the nurses are more attentive to disease treatment. However, psychological needs are as important as the physical manifestation. Study also PCCS 2006 SPEAKER’S ABSTRACTS November 3, 2006 identified that children undergoing cardiac surgery are at risk for developing Post Traumatic Syndrome Disorder (PTSD), especially if the ICU stay is prolonged. (Connolly, McClowry, Hayman, Mahony, Artman, 2004). Therefore, it is important to recognize children’s behavior and psychological stress, and teach parents ways to recognize children’s fear, emotion, and unusual behavior and inform health professional. The ongoing technological advances in cardiovascular critical care have improved the survival rates among children born with life-threatening congenital heart diseases. As technology progresses, nursing care needs to move beyond the technical surgical intervention, and try to respond to the physical and psychological challenges that parents face with their ill children. PCCS 2006 S33(1/4) TRANSCATHETER CLOSURE OF PDA : Some problems and difficulties SPEAKER’S ABSTRACTS November 3, 2006 Sukman T Putra, M.D., FACC, FESC Division Pediatric Cardiology, Department of Pediatrics & Child Health University of Indonesia, Dr Cipto Mangunkusumo, National Hospital Jakarta, Indonesia Transcatheter closure of persistent ductus arteriosus (PDA) with various occluders and coils has been well accepted as an alternative to surgical ligation . It decreases the morbidity, hospital stay and cost. Amplatzer duct occluder (ADO) is one of the devices available using for transcatheter closure of PDA which has been used worldwide with high success rate and safe. However, it is not recommended using ADO for infants less than 5 kg due to the risk of narrowing of the aortic arch by the devices. The use of ADO is well established and reported in many cardiac center all over the world. There has been various devices and coils were used since the first percutaneous closure of PDA performed by Portsmann. Some weakness of the previous devices are high incidence of residual shunts, sometimes complex delivery system, large delivery sheath and unsuitability for large PDA or small children. The incidence of residual shunting was reported between 3-38%. Several significant complication that may occur are embolization of the device and required surgery, acquired aortic narrowing, and blood loss. The procedural complications consist of two groups: mayor adverse events and minor adverse events. The mayor adverse events are device embolization requiring surgery or retrieved percutaneously, partial obstruction of pulmonary artery, loss of femoral pulses and bleeding that requiring transfusion. Whereas, the minor adverse events are hematoma of the groin, arrhythmia requiring medication, loss of peripheral pulses. In our multicenters clinical experiences over the last 3 years, 115 patients with PDA was occluded using Amplatzer occluder (92.2%) and coils (7.8%). The age ranging from 6 month to 13 yrs (median: 4.2 yrs) and the size of devices 4-12 mm (median 8mm). We had some problems in our case series. One patient a 6 yrs girl with large PDA(12mm) , implanted ASO (10mm) as the duct occluder was not available. The device embolized to RPA in 24 hrs follow up. Lung perfusion scan in 6 months revealed minimal uptake disturbances. Now clinically she was well, except mild tahypnoea. The other case a boy of 5,5yrs old with large PDA (12 mm), type C PDA (tubular) . ADO (14-16) implanted successfully, but embolized to RPA which was detected in 24 hrs follow up. Surgical duct ligation was performed with good results. We failed to implant duct occluder to a 5 yrs old girl with large PDA (11.2mm) . The surgical ligation was performed. But a significant residual shunt was detected in the follow up. The residual shunt was closed subsequently using Gianturco coil with good result. PCCS 2006 In conclusion, transcatheter closure of PDA is safe and effective as an alternative procedure to surgery . However, there were still some problems, difficulties and complications mostly related to the anatomy of the defects, size, weight and some procedures and techniques. Acknowledgements to : Dr Mulyadi M.Djer (Jakarta), Dr. Didik Haryanto (Jakarta), Dr Mahrus A.Rahman (Surabaya), Ria Nova (Palembang) on their great help in contributing the data. SPEAKER’S ABSTRACTS November 3, 2006 PCCS 2006 S33(2/4) Migrated stent and repositioning SPEAKER’S ABSTRACTS November 3, 2006 Seong-Ho Kim, M.D. Halla General Hospital, Jeju, Korea PCCS 2006 S33(3/4) VSD and AV block Transcatheter closure of perimembranous and subpulmonary VSD : immediate term follow-up for late AV block Background: Ventricular septal defect (VSD) is the most common congenital heart disease. Traditional surgical correction is the procedure of choice for closure of VSD. Transcatheter device closure using AmplatzerTM Perimembranous VSD device has emerged as an alternative treatment. Objectives: To report an immediate results with follow-up for patient underwent transcatheter closure of VSD. All cardiac rhythm pre and post procedure were recorded up to 6 months. Materials and methods: We collected data from patients who had transcatheter closure of VSD from February 2004 to June 2006. A peri-procedure ECG were reviewed in each patients for abnormalities such as right bundle branch block (RBBB) or complete 3rd degree atrioventricular block (3AVB) and follow-up ECG was done within 1 year post procedure. Results: 39 patients were included in this study. Their age varied from 1 to 34 years old (median age = 13.3 10.7 yrs). The average body weight was 38 25 kg. There were 34 pre-procedure ECG available for reviewed. 27 of them had normal ECG with 6 of them with RBBB and 1 of 1AVB with RBBB. There was successful transcatheter closure of VSD in 38 out of 39 patients. The diameter of VSD varied from 4.5 to 14mm (mean VSD diameter = 7.6mm.). The average device Size: VSD size (DV ratio) was 1.2 0.19. One patient did not have VSD device closure due to transient AVB. Type of VSD closed were Perimembranous VSD (n=28), subpulmonary infundibular type(n= 8), midmuscular (n =2) and right ventricular systolic pressure (RVSP) was 38 12 mmHg. Immediate post deploys 6 more patients developed RBBB post-procedure. Two of them had 3AVB out of hospital during the 1st week post-closure. One patient recovered after steroid treatment within one week (DV=1.33). The other had persistent 3AVB with symptom and had permanent pacemaker placed two months later (DV=1.4). Late 3AVB was noted in one asymptomatic girl (2 years old, DV=1.6). All three patients had perimembranous VSD. Conclusion : Transcatheter closure of VSD appears effective and relatively safe procedure. AVB was noted in 7.8% of patients. One permanent pacemaker was placed. Although there was no clear risk factors identifies. It appeared that the appearing of RBBB postprocedure may have some relationship with post-procedure early or late 3AVB. SPEAKER’S ABSTRACTS November 3, 2006 Kritvikrom Durongpisitkul, M.D. Division of Cardiology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand PCCS 2006 Predeploy Pre ECG SPEAKER’S ABSTRACTS November 3, 2006 34 Post deploy F/U Normal Normal Normal 27 24 33 RBBB RBBB 7 12 RBBB 3 3° AVB Unknown ECG 3°AVB 4 2 2 PCCS 2006 S33(4/4) Coil occlusion of VSD Nyuyen Lan Hieu, M.D. Vietnam Heart Institute, Hanoi, Vietnam SPEAKER’S ABSTRACTS November 3, 2006 PCCS 2006 S34(1/3) Controversial indications for pacemaker implantation in children SPEAKER’S ABSTRACTS November 3, 2006 Michael J. Silka, M.D. Division of Cardiology, Childrens Hospital Los Angeles, University of Southern California, USA In an effort to provide guidance for the clinician, the American College of Cardiology and the American Heart Association have developed guidelines (indications) for the implantation of pacemakers and implantable cardioverter-defibrillators. These guidelines are based on clinical data, which is often limited, and remain a topic of considerable debate. Three classes of recommendations exist: class I, where there is evidence and/or consensus that a pacemaker is generally indicated: class II, where there is controversy or debate regarding benefit of pacemaker implantation and class III, where there is evidence and/or consensus that cardiac pacing is not beneficial. In recognition of the controversy regarding some class II indications, these conditions have been further sub-divided into class IIA, where the majority of evidence is in support of pacing and class IIB, conditions where the usefulness of pacing is debatable. This session will examine the class IIA and class IIB indications, the current evidence regarding pacing in each of these conditions and possible future modifications to the existing recommendations. The specific class IIA indications to be discussed include: 1. The brady-tachy syndrome in post-operative congenital heart disease patients, particularly when chronic antiarrhythmic medications are required 2. Congenital complete heart block, beyond the first year of life, when the average ventricular rate is < 50/minute, there are abrupt pauses in the intrinsic rhythm or there are symptoms due to chronotorphic incompetence 3. The long QT syndrome with 2:1 AV block or complete heart block 4. Asymptomatic sinus bradycardia (<40/minute) in the child with complex congenital heart disease or when there are sinus pauses > 3 seconds 5. Patients with congenital heart disease and impaired hemodynamics due to sinus bradycardia or loss of AV synchrony The specific class IIB indications to be discussed include: 1. Transient postoperative third degree heart block that reverts to sinus rhythm with residual bifascicular block 2. Congenital third degree heart block in the asymptomatic child or adolescent with an acceptable rate and normal QRS complex 3. Asymptomatic sinus bradycardia (< 40/minute) in the child with prior biventricular repair of congenital heart disease or when there are sinus pauses > 3 seconds 4. Neuromuscular disease with any degree of AV block, with or without symptoms, due to the unpredictable progression of AV conduction disease PCCS 2006 S34(2/3) Cardiac Resynchronization Therapy in Children Mei-Hwan Wu, M.D., Ph.D. Departments of Pediatrics, National Taiwan University, Taipei, Taiwan SPEAKER’S ABSTRACTS November 3, 2006 Cardiac resynchronization therapy (CRT) using biventricular pacing may improve the myocardial performance in patients with electrico-mechanical dysynchrony. Typical patients are those with myocardial dysfunction and left bundle branch block. The effectiveness of CRT had been well documented in earlier eight landmark trials in adults with moderate to severe heart failure on optimal medical therapy. In children, potential benefits of CRT had been described in those with ventricular dysfunction and electrical delay (inter- or intraventricular dyysynchrony) both in the acute and chronic settings. In previous studies in children after surgery for congenital heart defects, short-term CRT by multi-site pacing may improve the postoperative cardiac output and systolic blood pressure and facilitate the weaning from cardiopulmonary bypass. As to chronic biventricular CRT in this age group, although the case number is still low, the benefit of CRT is well recognized. In contrast to adult patients, more than half of the children with CRT have associated congenital heart disease. Some have congenital complete atrioventricular block or dilated cardiomyopathy. The acute or chronic effects of CRT are shown not only in patients with two-ventricle circulation but also in those with single ventricle physiology or systemic right ventricle. Adverse effects are relatively common, as compared to adults, and mainly the coronary sinus lead issues. Case selection for CRT in children is usually based on the clinical criteria, including low ventricular ejection fraction and widen QRS duration (either left bundle branch block or right bundle branch block). In adults with dilated cardiomyopathy, it was estimated that 7-14 % of the patients had widen QRS duration and potentially benefit from CRT. In our series of children with dilated cardiomyopathy, 17 % of them had wide QRS duration (10 with complete left bundle branch block and 3 intraventricular conduction delay). Predictors for chronic CRT responders in children with ventricular dysfunction are still unclear. A relatively lower baseline ventricular ejection fraction had been found in responders as compared to non-responders. In adults, echocardiographic studies have demonstrated that the presence of LV dyssynchrony is an important factor determining response to CRT, whereas interventricular dyssynchrony appears of less importance. These criteria may also be adopted for children to predict the response after CRT but still need to be validated. PCCS 2006 S34(3/3) Pediatric AICD : A review SPEAKER’S ABSTRACTS November 3, 2006 Apichai Khongphatthanayothin, M.D. Chulalongkorn Hospital & University, Bangkok, Thailand PCCS 2006 S35(1/1) Long Term Outcomes in Patients with Congenital Heart Defects Bernadette McCormick, BSN, R.N. Rady Children’s Hospital, San Diego, CA. USA SPEAKER’S ABSTRACTS November 3, 2006 This presentation will review the current data on long-term physical and developmental outcomes of children with Congenital Heart Defects. The focus will be on patients with complex lesions. PCCS 2006 Debate1(1/2) Percutaneous Pulmonary Valve Implantation: Animal Studies and Human Experience SPEAKER’S ABSTRACTS November 4, 2006 Ziyad M. Hijazi, MD, MPH, FSCAI The University of Chicago Hospitals and The Pritzker School of Medicine. 5841 South Maryland Ave., MC4051 Chicago, IL 60637, USA Tel: (773) 702 6172; Fax: (773) 834-4100; E-mail: [email protected] Significant pulmonary valve regurgitation results in progressive right ventricle dilation that may lead to the risk of development of ventricular arrhythmias, right ventricle dysfunction and sudden death. The occurrence of pulmonary regurgitation and or obstruction is not uncommon after surgery for congenital heart defects, including tetralogy of Fallot, pulmonary atresia and any other surgical procedure requiring reconstruction of the right ventricle outflow tract. Even if a valved conduit or a bioprosthetic valve has been used for this purpose, progressive pulmonary regurgitation and or stenosis of such conduits or valves (homografts, Contegra, porcine valves) can occur. Surgical pulmonary valve implantation at an appropriate age may restore right ventricular function and improve the symptoms, however cardiopulmonary bypass and ventriculotomy needed for such operations may further impair the right ventricular function. Therefore timing and indications for resurrection of a competent pulmonary valve are controversial issues. In October 2000, Bonhoeffer and his colleagues reported the first animal experience of a percutaneously implanted pulmonary valve using a bovine jugular vein valve sutured inside an intravascular stent and since then the procedure has been reported in 59 patients. In December 2002, Cribier and his colleagues reported on the first human application of another percutaneous heart valve (PHV) in the aortic position. This valve was designed initially for application only in the aortic position and the early clinical experience with this PHV in the aortic position is ongoing. In this debate, I’ll discuss the animal experience implanting this valve in the pulmonary position and discuss our early human experience. Furthermore, I will discuss the experience with the Bonhoeffer valve that has been accumulated so far. He has done over 135 cases with very good results. PCCS 2006 Debate1(2/2) Transcatheter valve therapy (weird or wonderful?): Con Andrew N. Redington (Canada) The Hospital for Sick Children, Toronto, Canada SPEAKER’S ABSTRACTS November 4, 2006 PCCS 2006 S36(1/2) Natural History of Subpulmonic VSD and Echocardiographic Evaluation SPEAKER’S ABSTRACTS November 4, 2006 Layangool T., Kirawitthaya T, Sangtawesin C Queen Sirikit Institute of Child Health, Bangkok, Thailand Objective: To study the natural history of the ventricular septal defect, types of VSD, the incidence and onset of aortic valve prolapse (AVP) and aortic regurgitation (AR). Study design: A prospective cohort study. Population: The children diagnosis of isolated VSD who under one year of age at Queen Sirikit national institute of child health. The studying period was started from October 2000 to September 2006. By using Toshiba (Provision) or Aloka echo machine, echocardiographic studies were schedule performed every 2-3 month in the first year of age and then every 6 month to evaluate the size, location, flow across VSD, aortic valve morphology and evidence of aortic regurgitation. Results: Three hundred twenty one cases of VSD have been followed up. One was excluded due to associated hypoplastic RV. The overall 2,644 echocardiograms had been performed. The percentage of perimembranous, subpulmonic, muscular, inlet and multiple types were 70.3%, 19.4%, 5.6%, 3.1% and 1.6%, respectively. Five cases (1.6%) had loss to follow up. The incidence of AVP in subpulmonic VSD was 87.1% compared to 16.4% in perimembranous VSD, with a risk ratio of 5.3. From the survival analysis, AVP in subpulmonic VSD developed within 60 months of age; with nearly 80% had AVP in about 24 months of age. The incidence of AR in subpulmonic VSD was 37.1% compared to 5.3% in perimembranous VSD, with a risk ratio of 7.0. From the survival analysis, about 47% of subpulmonic VSD had AR occurred within 60 months of age, 25% of subpulmonic VSD had AR in about 30 months of age. The percentage of small, moderate, and large VSD was 62.5%, 15.9% and 21.6%, respectively. One hundred and three cases (32.2%) underwent cardiac operation. All, except one had VSD closure. The indications for VSD closure were congestive heart failure unresponsive to medical treatment or occurrence of AR. Fifty percent of subpulmonic VSD required surgical closure compared to 27.5% of the perimembranous type during the follow-up period. Sixty one cases (19.1%), including two cases of subpulmonic type had spontaneous closure of VSD. Progressive subpulmonic stenosis was found in ten cases (3.1%). Forty four cases (13.7%) of all VSD had associated minor cardiac anomalies. Five cases died during the follow-up period. Conclusion: The prevalence of subpulmonic type is relatively high in Thai children with VSD. This type of VSD has much higher incidence of AVP and AR compared to other types. The results of our study suggest that the aortic valve complications in subpulmonic VSD can develop in early childhood period. PCCS 2006 S36(2/2) Subpulmonary VSD – When and How to repair the AR? Katsuhiko Tatsuno, M.D. Department of Cardiovascular Surgery Chiba Cardiovascular Center, Ichihara Chiba, Japan SPEAKER’S ABSTRACTS November 4, 2006 Wide-ranging studies on the ventricular septal defect with aortic regurgitation (VSD with AR) started in 1950’s and 1960’s by Scott, Keck, Nadas, Halloran, Van Praagh and other many investigators elucidated the entire clinical and morphological features of this entity. Van Praagh (1968) explained that the normal aortic valve was supported by three parts, from above, at the leaflet level, and from below. In subcristal (perimembranous) VSD, the most relevant cause of AR was underdevelopment of one aortic commissure (lack of support from above). On the other hand, in subpulmonary VSD, AR was caused by lack of supportfrom-below with conal (infundibular) septum. We observed precise movement of the prolapsed aortic valve into VSD with thoracic aortography (Tatsuno 1973). This study confirmed that the anatomically unsupported aortic sinus and annulus were protruded by a left-to-right shunt blood stream through the VSD in the systolic phase. And intraaortic pressure in the diastolic phase forced to hang down the free margin of the aortic cusp resulting in AR. The first successful operation was made independently by Garamella and Starr in 1960. In the early periods, selection criteria of operative procedure were considerably confused. Robinson mentioned that VSD closure should be selected in the first step of surgical treatment. Spencer and Blumenthal reported superiority of the aortic valvuloplasty. On the other hand, Ellis, Dubost and Gonzalez-Lavin emphasized the necessity of a monocusp or total aortic valve replacement. After the initial confusion, Plauth(1965), Frater(1967) and Trusler(1971) introduced new techniques of aortic valvuloplasty. They placed a stitch in the three Arantius bodies of the cusps, and plicated the redundant free margin in order to keep apposition with the adjacent normal cusps. Following the improvement of surgical technique, aortic valvuloplasty became the acceptable choice for treatment of the prolpasing AR. While these surgical techniques were developing, Plauth, Hallidie-Smith (1969), Treasure (1971), and Trusler (1973) proposed the following criteria for selection of surgical treatment. 1) In the young patient with mild AR, VSD closure alone is indicated. 2) In the young patient with moderate or severe AR, aortic valvuloplasty is the first choice. However, if it was failure, a secondary operation should be prepared. 3) In the adult patient with severe AR, aortic valve replacement is selected. We proposed new criteria in 1973 for selection of surgical procedures based on the three determinant factors, 1) elapsed time after the onset of AR, 2) mechanical mobility of the bulging aortic valve during cardiac cycle, 3) severity of AR. PCCS 2006 SPEAKER’S ABSTRACTS November 4, 2006 Early detection of the prolapsed aortic valve and closure of the VSD, if possible before occurrence of AR, is another ultimate approach to eliminate this combined disease. If echocardiography and/or thoracic aortography are applied in all patients with subpulmonary VSD, and if a suitable surgical correction in the patient with prolapsed aortic valve, a good half of the cases of VSD with AR may be prevented. PCCS 2006 S37(1/2) The essence of isomerism of the atrial appendages, and its relationship to visceral heterotaxy Andrew Cook, M.D. Institute of Child Health, University College London, UK SPEAKER’S ABSTRACTS November 4, 2006 PCCS 2006 S37(2/2) Arrhythmia in Heterotaxy Syndrome SPEAKER’S ABSTRACTS November 4, 2006 Eun Jung Bae, M.D., Ph.D. Associate professor Department of Pediatrics, Seoul National University College of Medicine, Korea Heterotaxy syndrome is uncommon congenital disorder characterized by abnormal visceroatrial situs with either right isomerism of atrial appendages or left isomerism of atrial appendages. In heterotaxy syndrome, the structure of the cavo-atrial junction and atrio-ventricular junction is usually anomalous. In right isomerism, bilateral superior vena cava, bilateral sinoatrial nodes, and paired (twin) AV node may be found. In left isomerism, there may not be specific structure of sinoatrial node. Supraventricular tachycardia is common in patients with right isomerism in both preoperative and postoperative period. Abnormal electrophysiologic characteristics of AV junction and twin AV node may be the basis for this tachycardia. Recently we reported the high correlation of right isomerism and internodal tachycardia circling twin AV node. Among complex univentricular heart requiring Fontan type palliation, both heterotaxy syndrome and complete AV canaseptal defect were found to increase the risk of having a twin AV node. The presence of a rapid conducting bundle between the two AV nodes (M?nkeberg’s sling) was also documented by electrophysiologic recordings. Junctional tachycardia with AV dissociation is another supraventricular tachycardia occurring in heretotaxy syndrome. We also reported the electrophysiologic characteristics of this atypical focal junctional tachycardia. The patients with heterotaxy syndrome, especially in left isomerism, have high probability of developing bradyarrhythmia such as sinus bradycardia and AV block as well as tachyarrhythmia. In the case of heterotaxy syndrome, surgical method of neither lateral tunnel Fontan operation nor extracardiac conduit Fontan operation had significant influence on postoperative sinus node dysfunction . PCCS 2006 P4(1/3) Towards the Optimum Care of Pediatric and Congenital Heart Disease in the next decade. Roger B. B. Mee, M.D. Chairman Department of Pediatrics and Congenital Heart Surgery, Cleveland Clinic, USA (Retired) SPEAKER’S ABSTRACTS November 4, 2006 For the most part, the next 10 years of Pediatric and Congenital Heart disease management will see incremental improvements in outcome, institution by institution. There remains a wide range of results in terms of morbidity and mortality and this will persist, but one expects an increasing number of centres with near optimal results - a consistent 30 day or Hospital mortality of less than 2% and an incidence of pre-operative seizures in neonatal surgery of less than 5% The pathway to improving results will be focused on improved training through establishing standards of education, particularly for the surgeons; through increased commitment in terms of increasing numbers of dedicated full time congenital heart surgeons and interventional cardiologists working in groups in centres with high volume loads; through planning and organization in terms of geographical clustering of facilities within an institution; through administrative set up and centre governance; and through leadership fostering of a sense of mission. Many congenital heart programs will remain disadvantaged in terms of late referral of patients and limited resources. However, a most difficult field of medicine will yield to improved organization, commitment and a sense of mission – changes which can be made really quite rapidly. This expensive form of medicine becomes less so with increased patient load, increased efficiency and above all when morbidity is reduced. A most useful guiding principle is to Place the Patient First under ALL circumstances, as a matter of “Top Down” policy. PCCS 2006 P4(2/3) The Future of Cardiac Catheterization SPEAKER’S ABSTRACTS November 4, 2006 Ziyad M. Hijazi, M.D., MPH, FSCAI The University of Chicago Hospitals and The Pritzker School of Medicine, USA Interventional therapy for congenital cardiac defects is becoming more acceptable. There are four major areas that will advance further over the next decade: Hybrid therapy, transcatheter valve replacement, VSD closure and Completion of Fontan operation in the cath lab. The hybrid therapy will focus mainly on doing the first stage repair in the cath lab. This stage consists of bilateral PA banding, stenting the PDA and possibly enlarging the atrial septum. I will discuss the technical aspects and share some results. The transcatheter valve replacement already has started in the pulmonary valve position. The Bonhoeffer valve made of bovine jugular vein and sewn inside a CP stent has been implanted in over 150 patients with very good results. I will discuss this valve and the Cribier Edwards valve made of a stainless steel stent with equine pericardium leaflets. VSD device closure can be done percutaneously in the cath lab or perventricularly in the operating suite. I will discuss these techniques and outline our results. Finally, I will discuss the Fontan completion in the cath lab. PCCS 2006 P4(3/3) Delivery of Care in Congenital Heart Programs: Past, Present and Future Andrew N. Redington, M.D. The Hospital for Sick Children, Toronto, Canada SPEAKER’S ABSTRACTS November 4, 2006 The First Asia-Pacific Congress of Pediatric Cardiology and Cardiac Surgery November 1-4, 2006 FP1 FREE PAPER LONG-TERM RESULT OF PATIENTS WITH ATRIAL ISOMERISM AFTER MODIFIED FONTAN OPERATION Hoashi T, Ichikawa H, Fukushima N, Ueno T, and Sawa Y Division of Cardiovascular Surgery, Department of Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, 565-0871, Japan Background. Due to the improvement of operative strategy and techniques, patients with atrial isomerism tolerate Fontan operation. However, the long-term outcome is still unknown. We investigated the long-term clinical feature of the patients with atrial isomerism after modified Fontan operation. Methods. Since 1954, preparatory palliative procedure for Fontan operation was performed in 58, and Fontan operation was performed in 25(43%). Among these patients, 11 patients were identified to be followed up for more than 5 years after the definitive operation. The patients’ records were reviewed and the long-term result were analyzed. Results. Mean follow-up period was 14 years (6-21). Ventricular morphology was right in 10 and left in 1. Nine patients had common atrioventricular valve (CAVV). Moderate to severe CAVV regurgitation was recorded in 3 patients. No patient had total anomalous pulmonary venous connection with pulmonary venous obstruction. Surgical procedure of definitive repair was intra atrial routing in 1, atriopulmonary connection in 2, total cavo-pulmonary connection with extracardiac conduit (EC-TCPC) in 5 and Kawashima in 3. Fenestration was placed in 3, and one of them has not been closed. Concomitant operations were a CAVV repair and a replacement. Seven patients (63.6%) survived, and all but one patient showed NYHA class1 without complication. The latest catheterization of all survivors showed mean pulmonary artery pressure was 11± 2.6 mmHg (mean± S.D.) and systemic ventricular end-diastolic volume index was 104± 37. The current echocardiogram showed systemic ventricle ejection fraction was 51.4± 6.5%. The oldest survivor is 34 years old. Univariate analysis identified absence of CAVVR (p=0.04), staged strategy (intermediate BCPS or bidirectional Glenn shunt) (p=0.02), and ECTCPC (p=0.02) as significant predictors of long-term survival. Conclusions. Seven out of 11 atrial isomerism patients after modified Fontan operation were long-term survivors, and all but one showed excellent long-term clinical course. FP2 The results of pediatric arch repair under normothermia without circulatory arrest There was no operative death. Postoperative peak pressure gradient across the aortic arch was 6 mmHg. Postoperative urination were 4.7, 2.7, 3.3, and 3.4 ml/kg/day at POD 0, 1, 2, and 3, respectively. Max serum creatinine was 0.7 mg/dl. Duration of peritoneal dialysis was 2.9 days. Output of pericard-mediastinal drainage tube were 21.4, 12.8, 6.7, and 4.2 ml/kg/day at POD 0,1,2,and 3,respectively. Duration of drainage tube insertion was 4.0 days. Duration of respirator was 5.0 days. There were no cereblo-spinal dysfunction and no re-exploration for bleeding. Pediatric arch repair under normothermia without circulatory arrest preserves renal and coagulative function with few neurological complications. We believe that this technique is beneficial especially in a patient with ductal shock and in a more complicated case such as HLHS. PAPER To minimize the risk of hypothermic circulatory arrest, we have performed pediatric arch repair under normothermia without circulatory arrest. Since July, 2004, 12 children (10 neonates and 2 infants) underwent an arch repair. Ages ranged from 3 to 183 days. Body weight ranged from 2.5 to 4.4 kg. Diagnosis were Coarctation (CoA)/ VSD in 9, CoA/double outlet right ventricle (DORV) in 1, CoA/aortic stenosis in 1, and CoA/ tricuspid atresia (TA,type 2c) in 1. Preoperative status of patients were ductal shock in 2, respirator in 7, and PGE1 infusion in 7. Emergent operation was required in all 10 neonates. Bicaval cardiopulmonary bypass (CPB) was established through 2 cannulae at innominate artery (3.0 or 3.5 mm ePTFE graft interposed) and descending aorta (DesAo) just above the diaphragm. Arch repair was performed with end to end anastomosis in all but 1 case, in which Norwood type arch repair was required (CoA/TA). Concomitant procedures were VSD closure in 9, inter-ventricular rerouting in 1 (CoA/DORV), PA-debanding in 1, and modified BT shunt in 1 (CoA/TA). FREE Junichi Koizumi, Ishihara K, Mitsunaga Y, Takahashi K, Kazui T, Kin H, Ohsawa A, Oyama K, Kadosaki M, Kawazoe K Department of Cardiovascular Surgery, Memorial Heart Center, Iwate Medical University FP3 FREE PAPER OFF-PUMP MIDLINE STERNOTOMY APPROACH IN AORTIC ARCH REPAIR IN NEONATES WITH SINGLE VENTRICLE Yoshimichi Kosaka, Asou T, Takeda Y, Kajihara N, Norimatsu T Kanagawa Children’s Medical Center An off-pump midline sternotomy approach was employed in the first stage palliation for coarctation of the aorta associated with single ventricle in neonates. With this technique, both pulmonary arteries were come in sight and thus could avoid migration of the pulmonary artery band (PAB . Furthermore, the anomalous arch vessel could be repaired concomitantly. The purpose of this study was to review outcomes of the present technique. Patients: Five neonates had a functionally single ventricle associated with coarctation of the aorta. The distal aortic arch was hypoplastic in 4 and the coarctation was localized in the aortic isthmus in 1. One patient had the aberrant right subclavian artery. The median age at surgery was 10 days and the median body weight was 2.6 kg. All underwent the first stage palliation including aortic arch repair and PAB through a midline sternotomy without use of a heart-lung machine. Technique: The hypoplastic distal aortic arch was augmented with the left carotid arterial flap through a midline sternotomy. Thereafter, the aortic arch was reconstructed with an end-to-end anastomosis and the pulmonary artery was banded. In a patient with the aberrant right subclavian artery, it was divided at its origin of the aorta and pulled behind the esophagus and re-anastomosed to the right carotid artery in an end-to-side fashion. Results: There was no operative morbidity and mortality. All were extubated within 48 hours. Postoperative catheterization revealed no residuals in the aortic arch. All patients completed the second stage palliation, bidirectional Glenn shunt (BCPS). In two patients, subaortic stenosis developed and, hence, Damus-Kaye-Stansel anastomosis was performed concomitantly with BCPS. Total cavopulmonary connection was completed in two and awaiting in the remainder. Conclusions: An off-pump midline sternotomy approach was safe and useful in aortic arch repair in neonates with single ventricle. FP4 Mid-term results of bilateral pulmonary artery banding strategy for hypoplastic left heart syndrome PAPER Background: We report our experience of performing bilateral pulmonary artery banding (BPAB) for stage I palliation and maintaining systemic flow by prostaglandin E1 infusion or a main pulmonary artery to the descending aorta shunt until stage II palliation. Methods: Since 2002, BPAB for stage I was performed for eight hypoplastic left heart syndrome patients; 5 diagnosed with aortic atresia/mitral atresia, and 3 with aortic stenosis/mitral stenosis. Two patients had an associated chromosome anomaly. One had an intact atrial septum and left atrium-innominate vein communication and another had anal atresia and left external ear atresia. Results: Balloon atrial septostomy was performed in four patients before stage I. BPAB (right or left circumference: 9 to 14) was performed at 2 to 19 days of age and 2.3 to 3.5 kg weight. In all patients, delayed sternal closure was not required at stage I. Systemic flow was maintained in 6 patients by prostaglandin E1 infusion, and by a main pulmonary artery to the descending aorta shunt in 2; two required an additional main pulmonary artery to the descending aorta shunt before stage II. Before stage II, balloon atrial septostomy was performed in 4 patients, and an atrial septal defect enlargement or creation was in one patient each. One patient was lost by sudden death. For 7 patients, bidirectional Glenn shunt with aortic arch and coronary flow reconstruction was performed in stage II (3 to 9 months of age, 2.9 to 4.7 kg weight). Two early death occurred due to postoperative pulmonary hypertension crisis. The other 5 patients are alive (5/8: 63%), 3 with a status of Fontan completion, and 2 waiting for Fontan completion. Conclusions: The outcomes of BPAB strategy for hypoplastic left heart syndrome were satisfactory. BPAB strategy may offer a staged surgical option for hypoplastic left heart syndrome. FREE Shin Takabayashi, Hideto Shimpo 1, Kazuto Yokoyama 1, Hiroyuki Ohashi 2, Yoshihide Mitani 2 1 Departments of Thoracic and Cardiovascular Surgery and 2Pediatrics, Mie University Graduate School of Medicine FP5 FREE PAPER Biventricle Repair of Double outlet Right ventricle with NonCommitted VSD Chung-I Chang, Jou-Kau Wang, Mei-Hwan Wu Department of Surgery and Pediatrics*, National Taiwan University Hospital Purpose: Surgical treatment of double outlet right ventricle (DORV) with non-committed ventricular septal defect (VSD) is still controversial in either biventricular or univertricular repair and challenging to the pediatric cardiac surgeon. Materials and Methods: From Oct 1996 to June 2005, fifteen patients of DORV with noncommitted VSD underwent biventricular repair. The age distribution is from 13 days to 23 year old (Mean 2.8 years old). The sex ratio of male to female is eight to seven. The associate anomalies included pulmonary stenosis and atresia 4, ECD 2, straddling of atrioventricular valve 3, juxtaposed atrial appendage 2, CoA 2, LV diventriculum 1, anatomical corrected malposition (ACM) 1, TAPVR 1. Nine patient had palliative procedure (shunt 4, PA banding 3, CoA repair 2). The definite surgical procedures divided into two groups. Group 1, five patients underwent arterial switch operation. Two of them had VSD enlargement. Group 2. Ten patients underwent intraventricular rerouting procedure. Six of them had VSD enlargement. Only one had extracardiac conduit. Results: There are three early mortality and no late mortality. Two of the mortality were neonates. The follow up period was 3 months to 7 years. The postoperative complications are AV block 1, residual LVOTO 3, (pressure gradient < 20 mmHg), residual PS 3 and mitral regurgitation 1. Conclusion: Biventricular repair is feasible in DORV and non-committed VSD with certain anatomical features. Precise preoperative evaluations will determined the type of the definite repair. FP6 Mitral valve repair in pediatric patients for mitral regurgitation PAPER Background: Mitral regurgitation is uncommon and features wide spectrum of morphologic abnormalities in pediatric patients. We retrospectively evaluated the midterm results of mitral valve repair for mitral regurgitation in pediatric patients. Material and Methods: Between December 1993 and February 2005, mitral valve repair was performed for mitral regurgitation in 33 patients aged less than 18 years. Mean age was 5.4 5.4 years (range 4 months to 17 years) and 6 patients (18.2%) were less than one year old. Mean body weight was 20.4 16.7kg (range 5.9 to 69.5kg). 17 patients had associated cardiac anomalies; ventricular septal defect was the most common(n=8). In regards to pathologic findings, there were annular dilatation(n=12), cleft leaflet(n=8), leaflet prolapse(n=6), short chordae(n=6) and so on. The most common method of repair was double orifice technique(Table 1). Mean follow up duration was 47.5 39.0 months(range 4.3-138months). Results: There was no early mortality. In terms of mitral regurgitation, 32 patients showed improvement in the degree of regurgitation after the first operation. Eight patients(24.2%) underwent reoperation, and five of them required mitral valve replacement. The causes of reoperation were regurgitation in five and stenosis in three. Among three ring annuloplasty cases, two have developed mitral stenosis. Four out of 14 double orifice cases required reoperation. The median interval from the first operation to reoperation was 5.5months (range 0-89.2months). One early and one late mortality occurred in the reoperation cases, and both of them were subject to mitral valve replacement. The 5-year survival rate and the freedom from reoperation were 93.3 4.6% and 76.1 8.2%, respectively. The 5-year freedom from mitral valve replacement was 83.6 6.7%. There was no significant risk factor for reoperation. Conclusions: Mitral valve repair for mitral regurgitation in pediatric patients resulted in acceptable early and midterm mortality and reoperation rates. Long term follow-up and further studies will be needed. FREE Hyung-Tae Sim, Dong-man Seo, M.D. 1, Tae-Jin Yun, M.D.1, Jung-Jun Park, , M.D.1, Sung-Ho Jung, M.D. 1 ,Ju-Yeon Uhm, R.N. 1 , Young-Hwue Kim, M.D.2, Jae-Kon Ko, M.D.2, In-Sook Park, M.D.2 1: Asan Medical Center, University of Ulsan, Department of Thoracic and cardiovascular Surgery 2: Asan Medical Center, University of Ulsan, Department of Pediatrics FREE PAPER Table 1. Operation methods Operation method Double orifice technique Cleft repair Focal Annuloplasty Papillary muscle splitting Ring annuloplasty Leaflet resection Wooler’s procedure New chordae formation Kay annuloplasty No. 14 7 7 4 3 3 2 2 1 FP7 Sinus Node Dysfunction Following the Repair of Partial Anomalous Pulmonary Venous Connection PAPER Objective:An atrial pedicle flap in the intra-atrial rerouting for the repair of partial anomalous pulmonary venous connection(PAPVC)0is expected to grow, resist infection,and last for the life span of the patient. However, this procedure may lead to postoperative sinus node dysfunction(SND). This retrospective study is to identify the cause of the incidence of SND after repair of PAPVC. Methods:The records of 22 patients undergoing repair for PAPVC between September 1991 and January 2006 were examined.Mean age at repair was 5.8 years(range,5 months to 17 years). Fifteen patients with PAPVC to superior vena cava were repaired using the caval division technique. Seven patients had PAPVC to the right atrium. Intra-atrial rerouting was performed with a right atrial wall flap in 14 patients(Group A) ,and with a patch(6) or direct suture(2) in 8 patients(Group B). Results: Follow-up averages 4.7 years(range, 2 months to 12 years).There were no hospital or late deaths.Postoperative echocardiograms showed that all patients had no evidence of superior vena caval or pulmonary venous obstruction.The thirteen patients(93%) in Group A and two patients(25%) in Group B had SND early postoperatively. Eight patients with SND at the discharge were all in Group A. Six cases of them have still SND late postoperatively. One of them required implantation of a pacemaker. Conclusions:In the atrial pedicle flap repair, the incision or suture crossing the crista terminalis have the potential to cause SND. However, intra-atrial rerouting with patch or direct suture maintains normal sinus node function. FREE Hiroaki Hiroaki, Yoshihiro Oshima, Masahiro Yoshida, Chikashi Shimazu, Hironori Matsuhisa, Tomonori Higuma, Yutaka Okita Kobe Children’s Hospital, The Department of Cardiovascular Surgery FP8 FREE PAPER EXCLUSION OF THE NON-FUNCTIONING RIGHT VENTRICLE Ji-Hyuk Yang, Jun TG1, Park PW1, Huh J2, Kang IS2, Lee HJ2 Samsung Seoul Hospital, Sungkyunkwan University School of Medicine 135-710 Seoul, Korea Department of Thoracic and Cardiovascular Surgery, Samsung Seoul Hospital Objectives: Non-functioning right ventricle (RV) not merely cause collapse of pulmonary circulation but may affect function of the left ventricle (LV). To prevent the deleterious effects, the right ventricle was excluded surgically. Materials and methods: Fourteen patients underwent right ventricular exclusion between Dec 2000 and Feb 2006 at our institution. The patients were divided into two groups; Group I (n=10) had hypertrophied RV associated with pulmonary atresia/intact ventricular septum (n=9) or tricuspid atresia (n=1), and group II (n=4) had dilated RV with Ebstein malformation. In group I, the median age at surgery was 5 (0.2 ~ 13.8) months and RV exclusion was performed during a cavo-pulmonary anastomosis (n=6) or systemic-to-pulmonary artery shunt (n=4). In group II, the median age at surgery was 29 (0.2 ~ 377) months, and the exclusion was performed during a Fontan procedure (n=2), cavo-pulmonary anastomosis (n=1), and Starnes operation (n=1). The tricuspid valve was closed either with patch or directly in all patients except one having tricuspid atresia. Additional procedures for RV exclusion were Gelfoam insertion into the cavity (n=8) in group I, suture plication of ventricular wall externally or internally (n=3), or free wall resection(n=1) in group II. Results: In group I, there was no mortality. Seven patients showed totally obliterated RV, 2 patients still had small communications between RV and coronary artery, and 1 had remaining RV cavity, which impairs LV diastolic function. In this patient, tricuspid valve had been closed directly without the use of Gelfoam because of rather sizable RV cavity. Fontan procedure was completed in 5 of the 10 patients. In group II, two patients died of ventricular arrhythmia and low cardiac output. Another patient still had large RV cavity. All these patients had had their RV wall been plicated. The other whose RV wall had been resected showed shrinkage of RV and an improvement in the motion of ventricular septum and LV function. Follow-up was completed in all survivors for a period of 3.6 to 66.7 (median 24.6) months. Conclusion : Exclusion of non-functioning RV can provides effective decompression of right ventricle, and subsequently left ventricle in a certain type of functional single ventricle. Although our experiences are small, it is advisable that hypertrophied RV should be obliterated with thrombotic material and patch, and dilated RV with free wall resection. Also, it would be easier for RV to be collapsed, if RV exclusion is performed earlier stage of palliation when the patient is expected to follow single ventricle correction F9 AORTIC VALVE REPLACEMENT IN CHILDREN – LATE REOPERATION DUE TO PATIENT PROSTHESIS MISMATCH IS UNLIKELY IN RHEUMATIC PATIENTS PAPER Background : Rheumatic heart disease (RHD) and congenital heart disease(CHD) remains the two most common cause of aortic valve replacement (AVR) in children. Implanting adequate size prosthesis is important to avoid patient prosthesis mismatch and re-operation. Annular enlargement may be required for implanting adequate size prosthesis. Methods and Patient profile: From Jan 2001 till July 2006. 41 patients underwent AVR with St. Jude medical regent mechanical heart valve prosthesis using standard cardiopulmonary bypass technique. 22 patients had associated mitral valve replacement. 36 patients were operated for RHD and 5 for CHD. In group A, 33 patients had predominantly regurgitant lesion (AR) and only 3 patients had pure stenosis along with severe mitral regurgitation (MR). All patients ingroup B had bicuspid aortic stenosis. Two of them had previous balloon Aortic valvotomy. Results: Patients with CHD needed early intervention than RHD group(10 ± 4.12 yrs Vs 15.53± 2.59). The mean size of valve implanted in RHD group was significantly larger (21.33± 2.59mm Vs 17± 0 mm , p < 0.005) than CHD group. As a result, the mean indexed effective orifice area was significantly larger (1.87± 0.29mm/m2 Vs 1.47±0.54 mm/m2 , p < 0.05) in RHD group. Only one patient with RHD (2.7%) needed aortic annular enlargement. Where as in CHD group, 3 patients (60%) had annular enlargement. Conclusion: In our study aortic valve replacement in children with RHD is predominantly due to AR. Implantation of relatively bigger size prosthesis with significantly larger orifice area was possible with very low incidence of annular enlargement compared to patients with CHD. Therefore late re –operation due to patient prosthesis mismatch is unlikely in rheumatic patients. FREE Biswajit Bandyopadhyay, Das Mrinalendu., Ashutosh Raghuvanshi. Rabindranath Tagore International Institute of Cardiac Sciences FP10 FREE PAPER Modified simple sliding aortoplasty for supravalvar aortic stenosis Dong-Man Seo, Hong-Ju Shin, M.D. 1, Jeong-Jun Park, M.D.1, Tae-Jin Yun, M.D.1, Young-Hwue Kim, M.D.2, Jae-Kon Ko, M.D.2, In-Sook Park, M.D.2 1: Asan Medical Center, University of Ulsan, Department of Thoracic and cardiovascular Surgery 2: Asan Medical Center, University of Ulsan, Department of Radiology 3: Asan Medical Center, University of Ulsan, Department of Pediatrics Background: The surgical approach for treating supravalvar aortic stenosis has evolved from plain patch technique to 3-dimensional patch repair. However, this 3-dimensional patch is complex, time-consuming procedure. We propose a modified simple sliding aortoplasty for supravalvar aortic stenosis without using foreign material. Material and Methods: Between June 2001 and December 2005, 7 children (mean age 7 years; range 3 to 12) with supravalvar aortic stenosis were surgically treated. Four patients had clinical features of Williams syndrome. Three patients had undergone right pulmonary artery angioplasty due to right pulmonary artery stenosis. One patient had undergone aortic valve commissurotomy due to bicuspid aortic valve. Results: There were no in-hospital deaths. No patient required repeat surgery. Cardiopulmonary bypass mean time was 66.3±15.8 minutes (range, 51 to 94). The mean cross-clamp time was 28.9±11.7 minutes (range, 19 to 52). All patients were discharged from the hospital with no problems. Patients were followed-up for a mean duration of 29.7±21.8 months (range 2 to 55). All patients were underwent postoperative echocardiography or computed tomographic scan, which showed good results of the surgical correction. Conclusions: For the patients presented in this study, the mid-term results of modified simple sliding aortoplasty have proved promising. Long-term follow-up is required to determine if this technique ultimately improves aortic anatomy significantly enough to decrease the incidence of postoperative aortic stenosis and insufficiency, and results in fewer operations. FP11 A new concept of infundibular rehabilitation in the correction of TOF: a more functional and anatomic approach PAPER Objective: Recent evidences of the importance of the infundibulum in TOF repair made us develop a new concept of infundibular rehabilitation. The early results of our experience are presented. Methods: From Sep.2001 to Sep.2004, 85 consecutive patients underwent total correction of TOF. VSD was closed through the RA in every case. Transannular patching(TAP) was performed in 60 patients(group I,70.6%) and in 25 patients no patching was done(group II,29.4%). TAP was performed by a new technique consisting of minimal damage to the transverse basal loop while producing effective mobilization of the pulmonic annulus and the infundibulum. In order to evaluate the right ventricular(RV) function, the degree of PR and RV dilatation by echocardiogram and the QRS duration by EKG were assessed. The results were compared with those of patients who did not undergo TAP. Results: There were no significant differences between the two groups in terms of age and body weight, but CPB time(group I,95.1±18.3min vs group II, 82.3±14.0min,p<0.01) and ACC time(group I,63.6±10.4min vs group II, 53.1±9.7 min,p<0.01) were both longer in group I. There was also no mortality, in either group. A total of 5 re-operations(5.9%) were performed; 3 in group I(5%) and 2 in group II(8%), all of which were for residual RVOT stenosis. As for the RV function, there were no differences between the two groups with regards to RV dilatation(group I,19/60 vs group II,7/25,p>0.7), QRS duration(group I, 101.5 ±20.6ms vs group II, 97.9±18.3ms,p>0.4) or the incidence of mild and moderate PR(less than mild;group I,56/ 60,93.3% vs group II,24/25,96%, moderate;group I,4/60,6.7% vs group II,1/25,4%). Furthermore, there was no case of severe PR in either group. Median follow up was 30.9months. Conclusions: The new concept of the infundibular rehabilitation was safe and promising, especially in excellent RV performance. For assessment of long-term RV function, further quantitative studies should be followed. FREE Dong-Man Seo, Jeong-Jun Park, M.D.1, Tae-Jin Yun, M.D.1, Hyun Woo Goo, M.D.2, Young-Hwue Kim, M.D.3, Jae-Kon Ko, M.D.3, In-Sook Park, M.D.3 1: Asan Medical Center, University of Ulsan, Department of Thoracic and Cardiovascular Surgery 2: Asan Medical Center, University of Ulsan, Department of Radiology 3: Asan Medical Center, University of Ulsan, Department of Pediatrics FP12 FREE PAPER Redo surgery after corrective surgery for Tetralogy of Fallot in the long term period Kozo Matsuo, Sugimoto K, Kabasawa M, Asano S, Murayma H, Tastsuno K. Department of Cardiovascular Surgery, Chiba Cardiovascular Center. Objectives: Long-term results after corrective surgery (CS) of Tetralogy of Fallot (TOF) are reported almost satisfactory. We reviewed TOF patients who required redo surgery over 10 years after CS to examine the timing of re-operation regarding preservation of cardiac function. Patients and Methods: In 12 TOF-CS patients, the age at re-operation was 31 ± 11 years and the interval from the CS was 23 ± 8 years. The lesions to be repaired were pulmonary stenosis or regurgitation (PS, PR) in 10 pts, peripheral PS in 5, RV outflow stenosis in 3, atrial fibrillation or flutter (Af, AF) in 3, ventricular tachycardia (VT) in 3, residual shunt in 2 and so on. RVEF and RVEDV were 39 ± 4.3 %, 170 ± 45 %N in PR group against 43 ± 6.7% , 105 ± 15 %N in PS group respectively. Serious arrhythmia was seen in 6 pts out of 8 (75%) associated with residual shunt or PR. For repair of RV-PA lesions, transannular patch was used in 4 pts, tissue valve in 3 and hand-made valved conduit in 2. Maze procedure was added in 2 pts and cryoablation in 3. In 7 patients (PS 3, PR 4) RV muscles were sampled during re-operation to analyze histological changes. Results: There was no operative deaths and all patients are in I - II class of NYHA in the mean follow-up period of 48 months. CONCLUSION: RV dysfunction and arrhythmia were prevalent among the patients with PR or residual shunt. Histological examination of RV muscles indicated tendency of lighter degeneration in PS and earlier redo group. We conclude that early repair of residual shunt or PR should be considered to prevent secondary lesions in follow-up of TOFCS. FP13 Analysis arterial switch operation results of 113 patients with complete transposition of the great arteries PAPER Objective Aim to review and analysis the surgical results of 113 arteries switch operations. Methods 113 patients had been repaired by arterial switch operation from Jan. 2001 to Des. 2005. There were 60 patients with transposition of the great arteries and intact ventricular septum (TGA/IVS), 53 patients with transposition of great arteries and ventricular septal defect (TGA/ VSD) .The lowest body weight was 2.3kg, and the youngest operative age was 6 hours. The arteries Switch operation was performed underwent deep hypothermic circulation arrest and low-flow perfusion. Results The total mortality was 9.73%. There were 5 deaths among TGA/ IVS (8.3%), 6 deaths among TGA/VSD (11.3%) . Following improvement of surgical technique, post-operative management and cardiopulmonary bypass , the operative mortality was decreased from 16.65% to 5.5%. Conclusions The main reason for operative mortality was abnormal coronary arteries. The incidence of abnormal coronary arteries was high at TGA/VSD. The surgical results was not infected by the position of great arteries. The low cardiac output was appeared if the ratio of left ventricular pressure and right ventricular pressure less than 0.6. FREE Xu Zhiwei, Liu Jinfeng, Zhang Haibo, yanqing, Zhen Jinghao, Qiu Lisheng, Wang Shunming, Su Zhaokang , Ding Wenxiang. Department of Thoracic and Cardiovascular Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University, Shanghai 200127, China FP14 FREE PAPER Aortic translocation in the treatment of transposition of the great arteries with ventricular septal defect and pulmonary stenosis Xu Zhiwei, Wang Shun-ming, Zheng Jin-hao,Su Zhao-kang, Ding Weng-xiang Department of pediatric thoracic and cardiovascular surgery, Shanghai Children’s Medical Center Objective: Aim to review the clinical treatment of the aortic translocation operation for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis ( TGA/ VSD/PS ) Methods Between Augest 2004 and July 2005, Six patients with TGA/VSD/PS were surgical repaired by the aortic translocation operation. There were 5 male and 1 female. The operative age was from 4 month to 2 years (median age 11.33±6.86 month ) and the weight was from 5.6~11 kg (median weight 8.43±2.19Kg ). Results The median total bypass time was 153.83±33.70min (range from 112~204min ), and the median cross-clamp time was 89.16±18.61min (range from 73~139min ). There was no hospital death. All of patients were followed up from 2 month to 1 year and all patients are alive. The chest X- ray showed that the heart was a little enlargement than that of pre-operation and the pulmonary vascular marking was increased. ECG showed sinus rhythem There were trivial aortic insufficiency in two patients and mild aortic insufficiency in one patient. The ventricular function was normal, EF 74~86, FS 34~52. Conclusions Now the Rastelli repair has become the standard surgical repair for the treatment of TGA/VSD/PS, but the long-term results have been less than optimal. The aortic translocation and biventricular outflow tract reconstruction is a valuable procedure to prevent of left ventricular outflow tract obstruction and extra-cardiac conduit obstruction after Rastelii operation. It is need to accumulate experience of this new operation because of no many patients and a short period of followed up at this report. FP15 Arterial Switch Operation for TGA with Unusual Coronary patterns PAPER Introduction: Complex coronary anatomy is a risk factor for arterial switch operation (ASO). Its presence doesn’t preclude the feasibility of an ASO. Successful coronary translocation can be achieved in almost all cases of TGA with unusual coronary patterns by individualizing and using modifications of the standard techniques albeit at a slightly higher risk considering the unfavorable alternatives. Materials and Methods: The data of 157 consecutive patients who underwent ASO from June 2001 to July 2006, were analysed retrospectively. Of these 52 (33.1 %) had coronary pattern other than 1 LCx, 2R. The pattern of coronary arteries were circumflex coronary artery (Cx) from right coronary artery (RCA) in 29 patients, single coronary artery from sinus 2 (11), inverted coronary arteries (4), left anterior descending artery (LAD) from RCA arising from sinus 2 (3), RCA from LAD arising from sinus 1 (3), and dual circumflex coronary artery (2).Various techniques used to transfer theabnormal coronaries, were the standard button technique (15), medially based trap door incision (29), pericardial hood augmentation of the trap door technique(8), pericardial tubes of short length(3), and a Westaby like flap with pericardial patch augmentation (1). Nine patients required intra operative revision of the coronary anastomosis, as coronary flow was thought to be suboptimal during rewarming or weaning from Cardiopulmonary Bypass. Results: There was one operative death and three early postoperative deaths attributable to coronary perfusion related issues. All other patients had an uneventful recovery and had no regional wall motion abnormality on echocardiogram. On follow up (1 – 50 months Median 37 months), there were two late mortalities, one due to an unrelated cause, the other due to a possible coronary cause. All other patients (except two lost for follow up) have clinical and echocardiographic evidence of good coronary perfusion. Conclusions: 1. ASO should be performed in all patients of TGA with unusual coronary patterns, even though at a slightly higher risk, as the alternatives are dismal in the long term, especially when VSD is an associated lesion. 2. The technique of transfer should be individualized depending on the coronary anatomy and great artery relationship as assessed intraoperatively. 3. Aggressive revision of coronary transfer if myocardial ischaemia is suspected, has always paid rich dividends. This assumes significance particularly in developing countries where ECMO / VAD facilities are hard to come by if needed to be instituted in an emergency. 4. Though intermediate term follow up appears promising, long term patency of these transferred coronary arteries needs to be evaluated in the light of current literature. FREE Rao Suresh G, Shivaprakasha K, Sunil G.S, Bafna Mahendra, and. Krishna Kumar R* Division of Pediatric & Congenital Heart Surgery & Pediatric Cardiology* Amrita Institute of Medical Sciences, School of Medicine, Kochi, Kerala, India FP16 FREE PAPER Surgical Treatment for Transposition of the Great Arteries with Posterior Aorta CHUNG-I CHANG M.D., Shu-Chien Huang, Ing-Sh Chiu, Yih-Sharng Chen, Jou-Kou Wang*, Mei-Hwan Wu* Department of Surgery and Pediatrics*, National Taiwan University Hospital, Taipei, Taiwan We reported six infants of TGA with posterior aorta during 1993 and 2001 five of which underwent arterial switch operation. The other died before operation. The age at operation ranged 8days to 5 months old. All of them received cardiac catheterization and balloon atrial septostomy before operation. Typical picture of ventricularterial discordance with aorta located posterior to the main pulmonary artery was delineated. All hearts had a blind pouch at right ventricular outflow tract (RVOT) just in front of the aortic root, which was thought the characteristic feature of the anomaly. We performed arterial switch operation without Lecompte maneuver in the five operated patients. The ventricular septal defects (VSD) were closed through the native aortic root after aortic transection. Four of them were doing well during follow-up except mild to moderate pulmonary stenosis. One had second degree atrioventricular block and obstruction of superior vena cava. He died 65 days later postoperatively. There is no late mortality. We concluded TGA with Posterior aorta could be successfully corrected by arterial switch operation during early infancy. VSD closure through native aortic root and no Lecompte maneuver were recommended. FP17 Results of One stage repair of Transposition complexes and arch obstruction Ji-Hyuk, Tae-Gook Jun, Pyo Won Park, June Huh, I-Seok Kang, Heung Jae Lee Department of Thoracic and Cardiovascular Surgery, 2 Department of Pediatric Cardiology Samsung Seoul Hospital, Sungkyunkwan University School of Medicine 135-710 Seoul, Korea 1 PAPER FREE Objectives: While the results of an arterial switch operation (ASO) have improved remarkably in recent years, the presence of arch obstruction continues to make this a surgical challenge. This study evaluates the surgical outcome of the patients with transposition complex and arch obstruction. Materials and methods: Between 1996 and 2005, 10 patients underwent surgery for transposition complex and arch obstruction at Samsung Seoul Hospital. The indication for ASO was Taussig-Bing heart(TBH) in 6 patients, transposition of great arteries(TGA) with ventricular septal defect in 3, and situs inversus, TGA with intact ventricular septum in 1. Combined arch obstruction was coarctation in 8 patients and interruption in 2. All patients underwent ASO and arch repair simultaneously. Arch obstruction was relieved during a period of selective cerebral perfusion in 8 patients or circulatory arrest in 2. The mean body weight at surgery was 3.5 0.4 days. Preoperative aortic valve size was 7.7±1.0 mm, and the ratio of aortic to pulmonic valve size was 0.64±0.07 on echocardiography Results: There was no mortality. Sternal closure was delayed in all patients, who were discharged at 21.1±9.2 postoperatively. During 20.7±20.3 months of follow up, no patient developed recoarctation or airway obstruction. One patient underwent intervention due to branch pulmonary artery stenosis. In 2 patients, right ventricular outflow tract (RVOT) had to be reconstructed because of the small neo-pulmonic valve. However, no statistical difference could be found between the preoperative valve size of these 2 patients and that of other patients. Conclusion: Simultaneous correction of transposition complex and arch obstruction had no detrimental influence on survival. However this condition continues to be associated with longer hospital stays and an incremental risk of developing RVOT obstruction associated with nogrowth of the valve, which could not be explained solely by the preoperative small valve size in our small experiences. FP18 FREE PAPER Quality of life in adolescents after the Fontan operation in comparison to ordinary adolescents Yu Mi Im, Kim H.S, Yun T.J, Park J.J, Seo D.M, Jung S.H, Jhang W.K, Kim Y.W, Park I.S, Ko J.K Congenital Heart Disease Center, Asan Medical Center, seoul, Republic of Korea Purposes : To assess the overall quality of life(QOL) in adolescents after the Fontan operation, and to seek for relative risk factors affecting the QOL in these patients. Methods : Twenty-three adolescents who had received the Fontan operation were enrolled in this study (Group I). Twenty-three age/sex-matched ordinary adolescents selected by randomized sampling were also included for case-control comparison (Group II). Data were collected and collated from May 1st to June 21st, 2006. Quality of Life Profile (Adolescent Version) was used for the quantification of overall QOL. Student t-test and Analysis of Variance were employed for the case–control comparison, and multiple linear regression was utilized for the determination of risk factors affecting the QOL in group I. Results : The mean overall QOL scores of group I and II were 0.13 ± 0.73 and 0.38 ± 0.61, respectively. The difference, however, did not reach statistical significance. Group I had lower mean QOL scores compared to group II in ‘Being’ and ‘Belonging’ domains. Mean QOL score in ‘Becoming’ domain was comparable between the two groups. General characteristics, such as school attendance, religion, sex, and the number of siblings, had no impact on ‘Satisfaction’ in group I. Number of operations that post-Fontan adolescents had undergone (t=2.411, p=.025) and number of medications they were taking at the time of investigation (t=2.602, p=.017) had a negative impact on ‘Satisfaction’ in group I. Conclusion : Overall quality of life in post-Fontan adolescents was comparable to that of ordinary adolescents. Number of operations that post-Fontan adolescents had undergone, and number of medications they were currently taking had a negative impact on ‘Satisfaction’ score in postFontan adolescents. Given the fact that post-Fontan adolescents are unique in terms of mental and physical states, it is mandatory to establish a special supporting system for these patients. FP19 OUTCOMES OF PATIENTS WITH RIGHT ATRIAL ISOMERISM PAPER Background: Hearts with right atrial isomerism (RAI) are usually combined with complex cardiac anomaly and their surgical managements are varied and challenging. We reviewed our experience of these patients. Methods: Between April 1991 and April 2006, 84 patients were diagnosed with right atrial isomerism. Of these, 75 patients underwent surgical treatment. Median age and body weight at initial operation were 5.7months (0-141months) and 6.2kg (2.1-29.5kg), respectively. Thirtyfive (46.7%) patients had total anomalous pulmonary venous return (TAPVR). Pulmonary outflow tract obstruction was identified in 63 (84%) and included pulmonary atresia in 23 (30.7%). Follow-up was possible in all patients with median follow up duration of 36.8months (0 day182months). Results: In-hospital mortality was 13.3% (10/75) and late mortality was 20% (13/65). The most common cause of in-hospital mortality was low cardiac output (n=5). Causes of late death were infection (n=5), pulmonary vein stenosis (n=3), shunt occlusion (n=3), and cerebral infarction (n=1). Biventricular repair was done in two patients with one death. The others were in the single ventricle physiology. Until now 40 patients (53.3%) received Fontan procedures. Overall survival estimates were 71% at 5 years and 53.7% at 10 years. Survival rates in patients without TAPVR were 88% at 5 and 10 years and in patients with TAPVR were 52.7% at 5 years and 20.1% at 10years. Risk factors for in-hospital mortality in univariate analysis were age less than 1 month (p=0.019), TAPVR (p=0.038), and no PS (p=0.048). Risk factor for late death was only TAPVR (p=0.001). Conclusions: This study showed that the early results of patients with RAI were not so bad, especially, in patients without TAPVR. During the follow up we should keep in mind the problems related to the asplenia, pulmonary vein stenosis, and shunt occlusion. FREE Sung-Ho Jung, Dong-Man Seo, M.D.1, Jeong-Jun Park, M.D.1, Tae-Jin Yun, M.D.1, WonKyoung Jhang, M.D.2, Young-Hwue Kim, M.D.2, Jae-Kon Ko, M.D.2, In-Sook Park, M.D.2 1 Asan Medical Center, University of Ulsan, Department of Thoracic and Cardiovascular surgery, Seoul, Korea 2 Asan Medical Center, University of Ulsan, Department of Pediatrics, Seoul, Korea FP20 FREE PAPER The role of intraoperative transesophageal echocardiography in predicting neo-pulmonary artery stenosis after arterial switch operation Yih-Sharng Chen, MD*; Ming-Jiuh Wang, MD, Shen-Ko Tsai, MD, Ph.D; Chung-I Chang*, MD, Ing-Sh Chiu*, MD, Jou-Ko Wang , MD, Mei-Hwan Wu , MD, Department of Surgery*, Pediatrics and Anesthesiology, National Taiwan University Hospital and National Taiwan University Hospital YunLin Branch, National Taiwan University College of Medicine Background : The arterial switch operation (ASO) is the preferred surgical procedure for transposition of the great arteries (TGA). Supravalvular stenosis of the neo-pulmonary artery (neoPS) remains the major complication for long-term follow-up. We prospectively investigated the role of intraoperative transesophageal echocardiography (TEE) in predicting development of neoPS after ASO. Methods and Results: Between August 1997 and August 2002, 49 infants less than 2 months old with TGA undergoing ASO were prospectively studied. The neoPS found by intraoperative TEE (TEEPS) was defined when the peak flow velocity was over 3 m/sec at the neo main pulmonary artery (neo-MPA) after ASO. Regular follow-up of the status of neo-MPA was performed with transthoracic echocardiography afterward. Reintervention including balloon angioplasty or reoperation was done if indicated. According to the set criteria, 21 patients (42.8%) were found to have TEEPS. In patients with TEEPS the probability of freedom from reintervention was 28% at one year and was 23% at 2 years of follow-up (p<0.0001). On the other hand, the probability of freedom from reintervention for PS was 92% at one-year follow-up and 78% at 2 years of follow-up and thereafter in patients without TEEPS. The hazard ratio of reintervention (angioplasty or reoperation) for patients with TEEPS to patients without TEEPS was 7.29 (p<0.0001). In addition, the time interval from ASO to reintervention was significantly shorter in patients with TEEPS than in patients without TEEPS. Conclusion: Findings of the intraoperative TEE in neonates and small infants undergoing ASO are very helpful in predicting the development of severe postoperative supravalvular stenosis of the neo-MPA, and intraoperative TEE could provide real-time monitoring for this issue. FP21 Centrifugal Pump in Cardiopulmonary Bypass and Temporary Ventricular Assistance PAPER Background: The centrifugal pump can be utilized for cardiopulmonary bypass, ECMO, or ventricular assistance. This non-pulsatile ventricular assist device (VAD) concept has been in existence since late 1970s, but is only recently more utilized in children. Compared to roller pumps, there is less trauma to red blood cells and less pronounced inflammatory response; in addition, centrifugal pumps are also advantageous in the event of air entrainment since the acrylic cones create an air trap (as bubbles accumulate in the middle of the vortex). There is also purported evidence that myocardial recovery may be superior to ECMO. Herein we report our experience with centrifugal pump for cardiopulmonary bypass (CPB) and left VAD in pediatric (weight<15kg). Materials and Methods: Since January 2004, 17 cases (weight< 15 kg) underwent CPB with centrifugal pump. Ten of these patients underwent second procedure. Also, five patients, 4 months to 4 years (1.78±1.65years) and 6 to 14.5kg (8.98±3.57kg), were affected with postcardiotomy hemodynamic failure and left VAD was used in all cases. The circuit included a centrifugal pump and pretreated tubing and cannulas. All patients were drained from left atrium and four of arterial cannula was placed in ascending aorta whereas one was put in left femoral artery. The flow rate were maintained 70-100ml/kg.min. Anticoagulation was obtained by continuous heparin infusion (5-30u/kg.min) adapted to activated clotting time between 160 and 200 seconds. Coagulation factors were maintained above 50%, hemoglobin above 120g/L, and platelets above 80,000/ml. Artificial ventilation was kept at the same level as before VAD support. All patients were treated with peritoneal dialysis (PD) because of oliguresis or anuresis before or during VAD support. Results: In CPB cases, all patients were weaned form the bypass successfully. Eight cases (80%) with second procedure showed hemoglobin urine, whereas clear urine was observed in each first operation case. Regarding to the VAD cases, after 40 and 74 hours support, two patients were recovered and weaned from the VAD, and discharged successfully. The other three cases were expired from bleeding, right ventricular dysfunction and neurological complication, respectively. Comments: Although the centrifugal pump is benefit to the blood trauma, 80% of the second procedures still showed the hemoglobin urine. It seems the sucker instead of the blood pump is the major cause of blood trauma. And the temporary centrifugal VAD appears to be one of the choices as a bridge to postcardiotomy recovery. Left VAD is an effective method for bridging to FREE Wei Wang, De-ming Zhu, Hong Chen, Zhi-wei Xu, Wen-xiang DING Department of pediatric thoracic and cardiovascular surgery, Shanghai Children’s Medical Center, Shanghai Jiao Tong University, School of Medicine, Shanghai (200127), P.R. China recovery of the heart function even in the infant and small children. The choice of the device should be decided according to the right ventricular function, lung function. Complications such as bleeding, infection and thrombolitics are not uncommon and should be treated conservatively. FREE PAPER Intensive care FP22 Association of intensity and duration of hyperglycemia with morbidity and mortality in a pediatric cardiac ICU Objective: To study the association of intensity and duration of postoperative hyperglycemia with mortality and morbidity in children undergoing cardiac surgery. Design: A retrospective cohort study. Setting: Patients and Methods : We reviewed 157 children ( median age 24 months) who underwent surgery for congenital heart disease. Association of peak blood glucose and duration of hyperglycemia with mortality, inotropy requirement and organ dysfunction was analyzed using chi square, Fisher’s exact test and logistic regression analysis. Association with ventilatory requirement, ICU and hospital stay were analyzed using Mann Whitney-U test. Results: Peak blood glucose >126mg/dl and >150 mg/dl occurred in 89.8% and 86% of children respectively but was poorly associated with mortality and morbidity. However, peak blood glucose >200mg/dl occurred in 90/157 ( 57% ) and was associated with longer ventilatory requirement (mean 56.17±12.62 vs 12.46±0.92 hrs, p< 0.001), ICU stay (mean 112.63±17.52 vs 59.06±11.73 hrs, p< 0.01), and hospital stay (12.12±2.19 vs. 7.430.79 days, p< 0.01); increased incidence of acute renal failure (11/90 vs. 0/67, p= 0.003 ), acute lung injury ( 9/90 vs 0/67, p=0.008) and higher inotrope requirement (p<0.05) compared to children with blood glucose <200mg/dl. Overall mortality being low ( 3/157 deaths) - no association with mortality was found. Increased duration of hyperglycemia was strongly associated with MODS ( p< 0.05) and significantly increased epinephrine(p<0.01), norepinephrine (p<0.01) and milrinone requirement (p< 0.0001). Conclusion: Hyperglycemia > 126mg/dl was common in children who underwent cardiac surgery. Peak blood glucose >200mg/dl and prolonged duration of hyperglycemia is associated with increased morbidity. A prospective randomised trial of strict glycemic control is warranted. PAPER Hyperglycemia in sick adults is a known risk factor for adverse outcome. Data in pediatric patients is scarce. FREE Parvathi IYER, Sheth K, Kaushal SK, Dagar KS, Iyer KS Escorts Heart Institute & Research Centre W IT H D R A W N FREE PAPER FP23 FP24 Use of inhaled Prostacyclin analog (Iloprost) on the Pulmonary vascular reactivity in patients with Pulmonary arterial hypertension secondary to Congenital Heart defects: Randomized Controlled Trial PAPER Objective: To determine the efficacy and safety of inhalational or aerosolized prostacyclin analog (iloprost) on patients with pulmonary arterial hypertension secondary to congenital heart disease. Methods and Results: A prospective, randomized placebo-controlled interventional study was done on 27 pediatric patients ages 1-19 years old with congenital heart disease who underwent cardiac catheterization for hemodynamics studies. Patients were randomly assigned to receive either placebo or aerosolized iloprost 25 ng/k/min for 10 minutes(N=12). Baseline and postinhalational hemodynamic parameters were determined for both groups. Aerosolized iloprost caused a decline in the mean pulmonary artery pressure by 7.8 ± 1.4 mmHg ( p<0.001 vs. baseline) and in the diastolic PA pressure by 5.33 ± 2.19 mmHg (p<0.001) Patients who received iloprost had a decreased in the pulmonary vascular resistance by 4.21 ± 7.84 woods unit (25% from the baseline) and Rp:Rs ratio declined by 0.19 ± 0.21 (p=0.01 vs. baseline) There was a significant effect in the mean and diastolic PA pressure (p<0.001) as compared to placebo. With inhalational iloprost, there was no systemic arterial hypotension noted and there was no significant change in the cardiac output (-0.12 ± 0.67 L/min; p =0.54 when tested against baseline) Conclusion: Inhaled iloprost exerted a selective pulmonary hemodynamic response, reducing the mean and diastolic pulmonary artery pressure 30 minutes after inhalation. Moreover, it decreased the pulmonary vascular resistance without significant systemic vasodilation. There were no adverse reaction to iloprost observed. Keywords: iloprost, pulmonary artery hypertension, congenital heart disease ,prostacyclin, pulmonary vascular resistance FREE MARITES FLORES, Mappala,V. Baldera J Casa ML Lopez, WL Philippine Heart Center, Quezon City, Philippines FP25 FREE PAPER Cardiorespiratory effect of pressure-regulated volume control ventilation in infants after surgical repair of complex congenital heart disease Limin Zhu, Xu Zhuoming, Chen Lin, Shi Zhenying, Xu Zhiwei, Liu Jinfen, Su Zhaokang Department of cardiothoracic surgery, Shanghai Children’s Medical Center, Medical College of Shanghai Jiao Tong University Introduction : Mechanical ventilation is essential in the perioperative period for the successful treatment of infants undergoing congenital cardiac surgery. Mechanical ventilation supports the patient’s respiratory function during much of the operative period, recovery from anesthesia, hemodynamic stabilization in the cardiac intensive care unit (CICU), and weaning to successful extubation. Positive pressure ventilation has both positive and negative hemodynamic consequences. High intrathoracic pressure decreases venous return to the heart while at the same time decreasing afterload to the left ventricle. These combined cardiopulmonary interactions have complex effects on cardiac output. Pressure-limited ventilation was mainstay in pediatric critical care in the past due to the inability of ventilators to deliver small tidal volumes consistently. Pressure-limited ventilation is particularly problematic in critically ill children with congenital heart disease (CHD) due to the inability to deliver a constant minute ventilation in patients undergoing rapid changes in lung compliance. Pressure-regulated volume control (PRVC) ventilation is a mode of ventilation now available in newer ventilators. This method delivers a controlled tidal and minute volume in a pressurelimited manner, using the lowest possible pressure, which is constant during the inspiratory phase. The decelerated gas flow is pressure and flow constantly vary, breath by breath, to achieve the preset tidal volume at a minimum peak inspiratory pressure (PIP). It is particularly useful in patients ventilated when there are rapid changes in lung compliance and airway resistance. Objective : The aim of this study was to compare how three modes of ventilation, specifically PRVC, volume control (VC) and pressure control (PC), affect cardiac output, airway pressure and oxygenation in infants after surgical repair of complex CHD. Methods : Fifty nonconsecutive infants with mean age (7.0±4.9) months and mean weight (6.3±2.0) kg underwent cardiac surgery in our institution were enrolled in this study. The diagnosis and operation of these patients were listed in table 1. Patients were admitted to CICU after complete surgical repair their cardiac defect and stabilized on a Siemens Servo 300 or Maquet Srevo i ventilator in VC mode. Two hours after operation, hemodynamic parameters, airway pressure and ventilator settings, and an arterial blood gas were obtained. Patients were then changed to PRVC and PC mode, who were changed back to VC ventilation finally. The tidal Diagnosis With increased pulmonary flow CAVSD, PH CoA, VSD, PH CoA, MR, PH D-TGA/VSD, ASD, PH DORV, VSD, PH (Taussing-Bing) IAA, VSD, PH IAA, AP W, PH L-TGA, VSD, PH PTA, IAA, PH TAPVC (Obstructive), PH With decreased pulmonary flow DORV, VSD, PS D-TGA/IVS, ASD, PDA D-TGA, VSD, PS PA/VSD PA/VSD (S/P RVOT patch) TOF cases Surgical procedure cases CPB 5 3 1 6 1 1 1 1 1 5 Repair CoA,VSD repair CoA repair, MV plasty ASO+VSD repair B-Ts+PAB’!staged ASO ASO+VSD repair IAA,VSD repair IAA, A-P window repair VSD repair Corrective repair Corrective repair 5 3 1 5 1 1 1 1 1 1 5 ON ON ON DHCA+DHLF DHCA+DHLF DHCA+DHLF DHCA+DHLF DHCA+DHLF ON DHCA+DHLF ON 1 4 1 3 1 15 Corrective repair ASO Lecompt repair Repair Repair Corrective repair 1 4 1 3 1 15 ON DHCA+DHLF ON ON ON 0N CAVC: Complete atrial ventricular septal defect, PH: pulmonary hypertension, CoA: Coarctation of aortic arch, VSD: Ventricular septal defect, MR: Mitral valve regurgitation D-TGA/VSD: D-transposition of great arteries with ventricular septal defect, ASD: Atrial septal defect, DORV: Double outlet right ventricle, IAA: Interrupted aortic arch, APW: Aortopulmonary window, L-TGA: L-transposition of great arteries, PTA: Persistent truncus arteriosus, TAPVC: Total anomalous pulmonary venous connection, PS: pulmonary stenosis, D-TGA/IVS: D-transposition of great arteries with intact ventricular septum, PDA: Patent ductus arteriosus, PA/VSD: Pulmonary atresia with ventricular septal defect, TOF: Tetralogy of Fallot, ASO: Arterial switch operation, B-Ts Blalock-taussing shunt, PAB: Pulmonary arterial banding, DHCA: Deep hypothermia circulatory arrest, DHLF: Deep hypothermia low flow PAPER Table 1 Diagnosis and operation of these 50 patients FREE volume, positive end expiratory pressure (PEEP), inspiratory time, and respiratory rate were preserved as the initial setting. All of the measurements were repeated 30 minutes after each mode. Nineteen patients who needed mechanical ventilation more than 24 hours were repeated this protocol 24 hours after operation. Results : The hemodynamic data, airway and ventilator parameters, and oxygenation data on operative day and day 1 after operation were summarized in table 2 and table 3. The tendency of these parameters in different ventilation modes was shown in figure 1 and figure 2. On operative day, there was a significant decrease in PIP and an increase in dynamic compliance, respiratory index (RI) and A-a DO2 in either PRVC or PC mode when compared to VC ventilation. But the increase of cardiac index (CI), stroke index (SI) and PaO2/FiO2 were only obtained in PRVC mode, while no significant increase of mean airway pressure (MAP) was found at the same time. The results of 24 hours after operation were similar to the operative day. Conclusion : Using PRVC or PC mode has the similar result of oxygenation parameters and airway and ventilator parameters, while the improvement of hemodynamic and PaO2/FiO2 was only obtained when using PRVC mode. Compared to VC mode, patient’s CI increased 6.05% and 6% at postoperative 2 and 24 hours with PRVC ventilation. So PRVC mode is safety and beneficial during the period after surgical repair of complex CHD in infants. FREE PAPER Table 2 Hemodynamic data, airway and ventilator parameters, and oxygenation data 2 hours after operation Hemodynamic LAP (mmHg) CVP (mmHg) CI (L/min.m2) SI (ml/m2) SRVI (dyne.s.cm-5) PVRI (dyne.s.cm-5) Airway and ventilator PIP(cmH2O) MAP(cmH 2O) C dyn (ml/cmH2O) Oxygenation PaO2/FiO2 RI A-a DO2 VC PRVC PC VC2 9.94 3.00 9.45 2.57 2.83 0.88 19.40 6.54 10.16 2.97 9.59 2.71 2.98 0.99* 20.50 7.25* 10.39 2.89* 9.73 2.74 2.87 0.95 19.89 6.84 9.87 2.93 9.94 2.47 2.83 0.97 19.92 7.19 1795.70 626.97 1805.35 691.17 1882.24 644.76 1862.10 734.19 373.93 352.76 352.71 321.97 372.65 392.87 371.94 410.26 18.73 5.45 6.12 1.58 15.98 4.68** 6.29 1.46 16.25 4.52** 6.44 1.58* 19.04 5.90 6.15 1.64 5.57 2.39 6.91 3.11** 6.80 2.95** 5.83 3.46 333.64 103.38 1.09 0.85 130.39 65.35 361.79 106.16** 0.94 0.73* 120.44 58.71* 344.24 100.76 0.93 0.66* 119.66 57.07* 339.17 98.38 0.97 0.85 124.12 57.52 *: P<0.05, **: P<0.01 Table 3 Hemodynamic data, airway and ventilator parameters, and oxygenation data 24 hours after operation Hemodynamic LAP (mmHg) CVP (mmHg) CI (L/min.m2) SI (ml/ m2) SRVI (dyne.s.cm-5) PVRI (dyne.s.cm-5) Airway andv entilator PIP(cmH2O) MAP(cmH 2O) C dyn (ml/cmH2O) Oxygenation PaO2/FiO2 RI A-a DO2 VC PRVC PC VC2 9.95 3.37 9.45 2.57 3.24 1.17 24.18 9.15 9.89 2.73 9.59 2.71 3.63 1.41* 26.10 9.61* 10.42 3.04 9.73 2.74 3.39 1.12 24.85 8.90 10.26 2.68 9.94 2.47 3.43 1.47 25.11 10.60 1508.46 407.71 1523.11 501.10 1481.96 473.84 1516.14 602.09 271.84 81.64 238.48 85.55 237.00 81.10 227.83 67.83 17.58 4.60 5.89 0.94 14.84 4.81** 5.89 1.79 15.21 3.74** 6.58 1.77* 17.53 4.31 6.26 1.79 5.70 2.42 7.38 3.67** 6.81 2.76** 5.80 2.55 343.50 104.38 1.05 1.00 117.92 61.84 367.78 102.57** 0.88 0.75 107.18 61.75* 343.45 97.49 0.97 0.63 118.36 56.02* 334.55 101.12 1.07 0.78 121.48 60.09 *: P<0.05, **: P<0.01 FREE PAPER Figure 1 Tendency of airway and ventilator parameters (A), oxygenation data (B) and hemodynamic data (C) in different ventilation mode 2 hours after operation Figure 2 Tendency of airway and ventilator parameters (A), oxygenation data (B) and hemodynamic data (C) in different ventilation mode 24 hours after operation FP26 FREE PAPER Rescue patients of severe enterovirus71 infection with flexible cardiovascular medication Jeng-Sheng Chang, Shiou-Jien Lin, Hung-Chih Lin, Bai-Hong Su, Ching-Tien Peng Division of cardiology+, China Medical University Hospital, Taichung, 404, Taiwan Ever since the spring 1998, the severe enterovirus71 (EV71) infection in Taiwan has resulted in fatality of 224 children, mostly under 3-years-old. Although mild EV71 infection causes only hand-foot-mouth syndrome or herpangina, a full-blown severe EV71 infection usually presents itself by 3 devastating stages as encephalomyelitis, hypercatecholamine and shock. Because of its rapid flip-flop changes between hypertension/tachycardia and shock during the later 2 stages, patients may be expired precipitously on fulminant cardiopulmonary failure. Between May 1998 and Jan. 2001, we experienced 8 mortality cases in this hospital, which accounts for 19.5% and 80% case fatality rates of the 41 severe EV71 patients and the 10 patients complicating with pulmonary hemorrhage/edema (PH/PE), respectively. Starting from Feb. 2001, we implemented a flexible cardiovascular medication protocol on these patients, stressing on uses of - blockers and multiple vasodilators during the hypercatecholamine stage to suppress the severe tachycardia and hypertension to < 170 beat-per-minute and < 90percentile arterial blood pressure for age, respectively. Also, multiple inotropic agents were used to combat shock. Thereafter, until Apr. 2004 a total of 45 patients were treated by this protocol. The EV71 infection was confirmed in every patient by RT-PCR study on specimens collected from the throat, anus, blood or cerebrospinal fluid. The case fatality rate declined remarkably to 2.2% in the 45 severe EV71 patients, as well as reduced to 8.3% in those complicated with PH/PE. Conclusion: the flexible cardiovascular medication protocol is effective in reducing the mortality rate of severe EV71 infection. FP27 Low Cardiac Output in Pediatrics After Cardiac Surgery: Predictor and Clinical Outcome PAPER The purpose of this study was to identify patients at risk for the development of low cardiac output syndrome (LCOS) in pediatric after cardiac surgery. Low cardiac output syndrome was defined as poor perfusion due to transient myocardial dysfunction. Methods: The patient characteristics that were independent predictors of low cardiac output syndrome were identified among 472 consecutive pediatric patients who underwent cardiac surgery at Harapan Kita Hospital between January 1, 2005, and December 31, 2005. Results: The overall prevalence of low cardiac output syndrome was 15.43 % (n = 73). Logistic regression analyses identified eight independent predictors of low output syndrome and calculated the factor-adjusted odds ratios associated with each predictor: (1) residual lesion (odds ratio 141.98); (2) complexity score (odds ratio 1.74); (3) CPB time (odds ratio 1.01); (4) preoperative ICU (odds ratio 8.51); (5) preoperative CHF (odds ratio 3.14); (6) bleeding(odds ratio 24.88); (7) arrhythmia (odds ratio 4.78); and (8) pulmonary hypertension (odds ratio 3.75). The operative mortality rate was higher in patients in whom low cardiac output syndrome developed than in those in whom it did not develop (39.72% versus 0.75%, p < 0.001). Mean basic complexity score was 6.25 with mortality rate 6.76, and the overall performance was 5.83. Conculsion: Compared to STS and EACTS, the performance of Harapan Kita was still lower. LCOS caused longer time of intubation time, ICU and hospital stay. There were eight independents predictor that can be used to predicts of LCOS in patient of Harapan Kita Hospital . Patients at high risk for the development of low cardiac output syndrome should be the focus of more intensive management to prevent the development of LCOS. Key words : Low Cardiac Output Syndrome, pediatric, cardiac surgery FREE Eva Miranda Marwali, Busro PW, Budiwardhana N, Koto CG, Indriwanto,Roebiono PS, Rahajoe AU, Harimurti GM, Fachri D, Rahmat J National Cardiovascular Center Harapan Kita Jakarta - Indonesia FP28 FREE PAPER MILRINONE IMPROVE HAEMODYNAMICS DURING AND AFTER CORRECTIVE SURGERY FOR CONGENITAL HEART DISEASES. Mohammed Sallam, department of cardiac anaesthesia Prince Sultan Cardiac Centre, Riyadh, Saudi Arabia Maintenance of adequate oxygen balance is one of the primary objectives when dealing with paediatric patients undergoing cardiac surgery. Cardiac output is one of the major components of oxygen delivery so that its maintenance is an important consideration. Due to preoperative cardiac lesion and myocardial dysfunction secondary to the events related to cardiac surgery and cardiopulmonary bypass, circulatory supports by pharmacological means is frequently required. Therefore inotropes and vasodilators are used to improve cardiac performance after cardiac surgery. Epinephrine, dopamine and dobutamine are commonly used inotropes; phosphodiasterase III inhibitors as milrinone have been introduced in clinical practice recently. Milrinone produces a positive inotropic effect with concurrent vasodilatation and little chronotropic effect and lacking the undesirable effects of catecholamine which includes an increase in heart rate and myocardial oxygen consumption, down- regulation of beta-adrenergic receptors and increase in systemic vascular resistance. As a result of these differences, milrinone has become a valuable tool in the treatment of infants and children following cardiac surgery. Several investigators have studied the effect of milrinone in reversing the low cardiac output frequently observed in infant and children after cardiac surgery (25% of cases develop low cardiac output syndrome after cardiac surgery). Chag and colleagues administered milrinone 50 mic.gm over 15 minutes in 10 neonates suffering from low output syndrome following cardiac surgery and observed an averages increase in CI of 42% and averages decrease in systemic and pulmonary vascular resistance of 37% and 27% respectively. Baily et al characterized the pharmacodynamics of milrinone along its correlation with the pharmacokinetics in 20 children between 3-22 months of age after they underwent repair of congenital heart defect. A loading dose of 50 mic.gm/kg given over a period of 5 minutes resulted in a mean decrease in mean arterial pressure of 12% and mean increase in cardiac index of 18% at a mean peak plasma concentration of 235 ngm/ml. Chu et al studied the effect of a loading dose of 20 mic.gm/kg followed by continuous infusion of 0.2 mic.gm/kg/min in 10 children with post bypass pulmonary hypertension after TOF repair within 6 months, they found a significant reduction in PAP/SBP within 15 minutes and the effect persist for 24 h. during infusion without remarkable adverse effect. PAPER About 750 cases/year of different types of congenital heart diseases are corrected surgically in our center (PSCC.), and we are using milrinone during surgery and in ICU in almost of them with good haemodynamics and less postoperative complications. FREE An important multicenter study done in USA (31 center participated in this study) evaluated the efficacy and safety of prophylactic milrinone in paediatric patients (about 238 patients) at high risk for developing low cardiac output syndrome, they used different doses of milrinone and they concluded that high dose milrinone after pediatric congenital heart surgery reduce the risk of low cardiac output syndrome. FP29 FREE PAPER Infliximab treatment for refractory Kawasaki disease in Korean children Song Min Seob, Yoon KL, Sohn SJ, Oh JH, Lee SB, KimCH Department of Pediatrics1, Paik hospital, Inje University, Pusan, Department of Pediatrics2 Eastwest Neo Medical Center, Kyunghee University, Seoul, Department of Pediatris3, Mokdong Hospital, Ewha Womans University, Seoul, Department of Pediatrics4, St.Vincent’s Hospital, Catholic University, Suwon, Department of Pediatrics5, Kyungpook National University, Daegu, Republic of Korea Introduction: This study is a multicenter study to evaluate the use of tumor necrosis factor (TNF) -alpha blokade for treatment of patients with refractory Kawasaki disease (KD) in Korean children. Refractory KD was defined as the persistence or recrudescence of fever or persistent arthritis after multipe dose of intravenous immunoglobulin or methylprednisolone pulse therapy with high dose aspirin. Methods: Six cases (5 M, 1 F; median age 2.4 years(0.3 to 5.4 yrs)) were retrospectively collected from clinicians throughout Korea between July 2004 and January 2006 who had used infliximab, a monoclonal antibody against TNF-alpha for patients with refractory KD. Results: Response to therapy with cessation of fever occurred in 5 of 6 patients including one patient with resolution of fever and accompanying persistent arthritis. C-reactive protein level was elevated in all patient before infliximab infusion, and the level was lower following infusion in all 5 patients in whom it was remeasured . There were no infusion reactions to infliximab but there was one case in which acute hepatitis occurred during acute treatment and calculous cholecystitis occurred after 4 months and subsided later. All patients had coronary artery abnormalities (from mild dilatation of coronary artery to aneurysms) documented by echocardiography before infliximab therapy. Two had transient mild dilatation of coronary artery and four had coronary aneurysms with subsequent normalization in two patients and persistent mild dilatation in one, aneurysm in one patient on follow-up echocardiography. It seems that no further significant progression of coronary artery lesions develop after infliximab treatment. Conclusion: These preliminary data in this small series of patients suggests that infliximab can be useful in the treatment of refractory KD. Multicenter trials with larger numbers of patients are necessary to assess it’s clinical efficacy and safety in refractory KD. FP30 FREE W IT H D R A W N PAPER FP31 Glycemic control in the pediatric cardiac ICU : is it useful ? FREE PAPER Parvathi IYER, K, Kaushal SK, Dagar KS, Iyer KS Escorts Heart Institute and Research Centre, ND, 110025, INDIA Hyperglycemia in the pediatric cardiac patient has been reported to be associated with increased morbidity and mortality. Data on glycemic control is not available due to concerns of insulin related hypoglycemia. Objective : To compare the effect of moderate glycemic control ( target blood sugars < 150mg/ dl and less tight glycemic control ( blood sugars 150 – 200mg/dl) on outcome in children undergoing cardiac surgery. Design : A prospective randomized controlled study. Setting, Patients and Methods : 302 children under 21 years of age undergoing cardiac surgery were randomized to receive insulin therapy to maintain blood sugars 1) < 150mg/dl or 2) 150-200mg/dl. The two groups were comparable wth regard to age, weight, type of surgery ( open/closed), PIM and Aristotle score, admitting and peak blood sugar levels. Insulin was administered along with potassium as an infusion to prevent hypokalemia. Differences in primary ( mortality) and secondary ( measures of morbidity) outcome were analyzed using Fisher’s exact test, chi square, Mann-Whitney U and student t test as appropriate. Results : Peak blood glucose >126mg/dl occurred in 98.34% admitted to the PCICU. Mortality was similar in both groups ( 2% and 0.7%). Organ failure ( p<0.05), multiple vasoactive and inotrope requirement ( p< 0.05), hydrocortisone requirement for vasopressor unresponsive hypotension ( p<0.01) were significantly greater in group 2. Group 2 also had longer ICU stay (108.94 hours vs 73.39 , p<0.05) and overall hospital stays (9.74 vs 7.78 days. There were no differences in the incidence of renal failure, acute lung injury, sepsis or severe sepsis in the two groups. Insulin related asymptomatic hypoglycemia ( blood sugar < 40)and hypokalemia (potassium < 3.0 mmol/L was infrequent – 3 and 4 occasions respectively. Conclusion: Level of glycemic control did not influence mortality -overall mortality being low. Preliminary observations indicate that relatively tighter glycemic control ( target blood sugars < 150mg/dl) was associated with reduced morbidity in children undergoing cardiac surgery. These observations need to be validated in larger studies or in multicentric trials. FP32 CORONARY ARTERIAL ABNORMALITIES AFFECT SURVIVAL IN PATIENTS WITH PULMONARY ATRESIA AND INTACT VENTRICULAR SEPTUM PAPER Background : Pulmonary atresia with intact ventricular septum remains a frequently lethal condition because of associated anomalies, though survival is improving. We suspect that the arterial fistulous connections (fistulae) between the right ventricular (RV) cavity and coronary arteries are a significant factor. Methods : Eighty-eight patients had surgical treatment between 1967 and 2002. The first procedure was a shunt alone in 45 patients, a shunt plus RV outflow enlargement in 34 and RV outflow enlargement only in 9. Among hospital survivors 19 have had a Fontan procedure, 7 had a 1.5 ventricular repair and 12 had a 2 ventricle repair. Cineangiocardiograms (angios) in 83 patients were available for review. The size of the tricuspid valve (TV) and RV, the presence of fistulae, their severity (graded as none, small, significant with opacification of the aorta from a RV angio, associated with one interruption of a major coronary or associated with two interruptions) were noted. A myocardial score quantifying the amount of left ventricular myocardium affected by coronary arterial occlusions was applied to evaluate the degree of RV dependent coronary arterial circulation. Results : Factors not affecting survival were the presence fistulae found in 58/83 (70 %, P = 0.25), and interruptions in 26 (31 %, P = 0.08). Significant factors included hypoplasia of RV (P = 0.04), severity of fistulae (P = 0.02), increased myocardial score (P = 0.005), and absent connections of coronary arteries to aorta in 11 (13%, P = 0.002). Conclusions : Awareness of the severity of the coronary arterial abnormalities should assist in planning patient treatment. FREE LOUISE CALDER and Occleshaw, CJ Green Lane Paediatric Cardiac and Congenital Services, Auckland, New Zealand FP33 FREE PAPER MAGNETIC RESONANCE CORONARY ANGIOGRAPHY AND MYOCARDIAL IMAGING TO EVALUATE CORONARY ARTERIAL LESIONS AND MYOCARDIAL ISCHEMIA IN PATIENTS WITH KAWASAKI DISEASE Atsuko Suzuki, Takemura A, Kitatsume T, Inaba R, Sonobe T,Tsuchiya K, Korenaga T (1) Department of Pediatrics, Tokyo Teishin Hospital, Tokyo 102-8798, Japan. (2) Department of Radiology, Tokyo Teishin Hospital, Tokyo 102-8798, Japan. (3) Department of Pediatrics, Japan Red Cross Medical Center, 150-8935,Tokyo, Japan We have performed non-contrast enhanced, free-breathing magnetic resonance coronary angiography (MRCA) in 470 patients with Kawasaki disease from the acute to the late phase (aged 4 months to 37 years; median 9.1 years ) by 1.5T Gyroscan Intera Master R.9 (Philips) since July 2000. The steady-state free precession technique (SSFP), and navigator-echo proton density-weighted black-blood imaging (BB), were both performed in 106 patients from May 2003 to July 2005. We evaluated the efficiency of MRCA in detecting obstructive lesions in the 106 patients based on X-ray coronary angiography. With SSFP, sensitivity of occlusion and localized stenosis was 94.2% and 97.2%, specificity was 99.5% and 97.2%, and the negativepredictive value was 99.5% and 97.2%, respectively. BB visualized a remarkably thick intima of aneurysms and/or thrombi in 38 lesions. Magnetic resonance myocardial imaging (MRMI) was performed in 22 patients with obstructive lesions from August 2005 using contrast agents (gadolinium), and it showed ischemic regions (n= 15) by perfusion defect and myocardial infarction (n=23) by late hyper enhancement. If the transmural extent of the hyper enhancement is less than 50% (n= 8) the myocardium is considered to remain viable. MRCA and MRMI are useful to follow up patients with coronary artery lesions and to determine the indication for bypass surgery. FP34 THE UTILITY OF CORONARYMAGNETIC RESONANCE ANGIOGRAPHY IN CHILDREN UNDER SIX YEARS OF AGE WITH KAWASAKI DISEASE. PAPER To follow up coronary arterial lesions due to Kawasaki disease (KD) using noninvasive magnetic resonance coronary angiography (MRCA), we studied a method to improve the quality of images in young children. Non-contrast enhanced, free-breathing MRCA with the vector ECG gating real-time navigatorecho 3D steady-state free precession technique (SSFP) was performed using a 1.5-T wholebody MR imaging system (Phillips) in 68 children with KD aged 4 months to less than 6 years.0 A flex medium coil was used. Data were acquired by the whole heart imaging technique. Slices, 1.8 mm thick (interpolated to 0.6 mm), were acquired with a 180 to 200 mm FOV and were reconstructed with a 512 360 matrix. Patients were sedated during the examination. Many parameters were optimized for each patient; i.e., field of view, acquisition delay, turbo-field echo-factor, navigator-window and resolution, which resulted in the acquisition of high-resolution and high-signal images of the coronary arteries. These conditions remarkably improved not only the quality of the images, but also the detection rate of coronary arterial segments (American Heart Association) in the children. The rates were as follows; Segments 1(97%), 2 (97%), 3(87%), 4 (66%), 5 (97%), 6 (96%), 7 (83%), 8 (56%), 9 (53%), 10 (21%), 11 (96%), 12 (29%), 13 (93%), 14 (54%), and 15(65%). MRCA is a useful method for evaluating coronary aneurysms from the early stages of KD, even in infants and small children. FREE Atsushi Takemura, Suzuki A, Kitazume T , Sonobe T , Tsuchiya K ,and Korenaga T 1) Department of Radiology, Tokyo Teishin Hospital, 102-8798 Tokyo, Japan.0 2) Department of Pediatrics, Tokyo Teishin Hospital, 102-8798 Tokyo, Japan. 3) Department of Pediatrics, Japan Red-Cross Medical Center, 150-8935 Tokyo,0Japan FP35 FREE PAPER The effects of ventilation level on cerebral blood flow after bidirectional superior cavopulmonary connection LU Ya-nan, LIU Jin-fen, SHI zhen-yin, Xu Zhi-wei, SU Zhao-kang, DING Wen-xiang. Department of Pediatric Cardiothoracic Surgery, Shanghai Children’s Medical Center, Xinhua Hospital, School of Medicine Shanghai Jiaotong Universitsy, Shanghai 200127, China Objective: In most instances, after congenital heart diseases surgery, hyperventilation can decrease the pulmonary vascular resistance, thus it can increase the pulmonary blood flow and may elevate the oxygen content in blood. But there is a clinical phenomenon that hyperventilation actually impairs, rather than improves, systemic oxygenation in patients after Bidirectional superior cavopulmonary connection. Because the connection places the cerebral and pulmonary vascular beds in series, the probable explanation for this effect is that hyperventilation produces hypocarbia, which increases cerebral vascular resistance and decreases cerebral blood flow. The aim of our study was to determine the effects of different ventilation level on cerebral blood flow after bidirectional superior cavopulmonary connection. Methods: Eight pediatric patients who underwent bidirectional superior cavopulmonary connection were enrolled in this study. There were six males, 2 females. Ages ranged from 8 months to 13 years, the median age was 14 months. All patients were diagnosed as functional single ventricle. The transcutaneous oxygen saturation was 60%-78%, mean 72%. Patients were studied in the intensive care unit, within 5 hours after the operation, while sedated, paralyzed, and mechanically ventilated. Initial ventilation parameters were set as synchronized intermittent mandatory ventilation, volume control mode. The tidal volume was 10-15ml/kg, ventilation frequency 20 per minute, inspiratory time 0.75S, positive end expiratory pressure 0mmHg, FiO2 40%-50%. Different ventilation level was achieved by adjusting mechanical ventilation frequency. Each patient was studied at 3 consecutive time points: (1) baseline, during normal ventilation; (2) during hyperventilation; (3) during hypoventilation. Blood gas analysis, hemodynamic parameters and Doppler determinations of internal carotid artery were obtained after at least a 20-minute stabilization period at each time point of the protocol. During the study the oxyhemoglobin in the cerebral tissue was continually monitored using near-infrared spectroscopy. Results: During hyperventilation, there were significant decreases in arterial pressure and superior vena cava pressure, the mean arterial partial pressure of oxygen and carbon dioxide both decreased significantly while the peak velocity of the internal carotid artery blood flow and the oxyhemoglobin in the cerebral tissue which represent the perfusion of the cerebral tissue was also decreased significantly. During the hypoventilation the opposite phenomenon were observed. Conclusion: The hemodynamic status, systemic oxygenation, cerebral blood flow and thus the pulmonary blood flow can be affected by ventilation level after bidirectional superior cavapulmonary connection. Compared with hyperventilation, hypoventilation improves systemic oxygenation and arterial blood pressure, the mechanism of these effects is supposed that hypoventilation decreases cerebral vascular resistance, thus increasing blood flow through cerebralpulmonary series after superior bidirectional cavapulmonary connection. FREE PAPER FP36 FREE PAPER Cardiac Conduction Disturbance Detected from a Large-Scale Pediatric Population Screening: Prevalence and the Implications Chiu Shuenn-Nan, Mei-Hwan Wu, Jou-Kou Wang, Chun-An Chen, Ming-Tai Lin, En-Ting Wu, Yu Chuan Hua, Hung-Chi Lue, Taipei Pediatric Cardiology Working Group National Taiwan University Hospital, and Medical College, National Taiwan University Objective: Data regarding the cardiac conduction disturbance (CCD) detected from school children survey are limited. We sought to obtain an estimate for the prevalence and the clinical implication of CCD. Patients and Methods: A citywide survey on elementary, junior and senior high school, by using questionnaire, 4-lead EKG, phonograph and physical examination, had been performed in Taipei from 1999 to 2001. Patients with abnormalities in each item were referred for final diagnosis. Results: From 432,166 EKG, the prevalence of CCD was 0.856% and was higher in male than in female (0.902% vs. 0.807%, p=0.001). Incomplete right bundle branch block (RBBB) (0.360%), complete RBBB (0.152%), ventricular premature beat (0.114%) and Wolff-ParkinsonWhite syndrome (WPW) (0.068 %) were the most common CCDs. Second-degree atrioventricular block (AVB), IRBBB, CRBBB and intraventricular conduction delay (IVCD) were more often found in male, but vise versa for atrial premature beat and long QT. The prevalence of CCD increased with age, as 0.589% in elementary and 1.079% in high school students. Detection of CCD identified patients with previously undiagnosed atrial septal defect (ASD, 63 patients). The positive predictive value of having ASD when EKG showed IRBBB, IVCD, and 1st degree AVB was 2.70%, 4.30%, and 2.37%, respectively. In addition, 15 high-risk cardiac patients were sort out in those with CCD (0.405%). Conclusions: The prevalence of CCD in children, commonly including IRBBB, CRBBB, VPC and WPW, was 0.856 and was lower than that in adults. The presence of CCD helps to detect unrecognized ASD and possible high-risk cardiac patients. FP37 REVERSIBILITY OF SEVERE PULMONARY HYPERTENSION AMONG PEDIATRIC PATIENTS WITH LEFT-TO-RIGHT SHUNTS USING MILRINONE – A DOUBLE-BLIND, PLACEBOCONTROLLED INTERVENTIONAL TRIAL PAPER Background : Preoperative assessment of pulmonary vascular reactivity among children with CHD specifically L-to-R shunts with pulmonary hypertension (PHT) is routinely performed to identify patients with fixed PHT who are at risk for heart failure and death after surgical correction. Several maneuvers aside from giving of 100% oxygen & nitric oxide, employs the use pharmacological agents to lower PVR have its own limitations and complications. Several studies already demonstrated the safety of Milrinone in the treatment of heart failure thus; it is the objective of this study to examine the feasibility of using Milrinone to test pulmonary vascular reactivity in pediatric patients with L-to-R shunt and severe PHT before the contemplated surgical correction. The hypothesis of this study is that Milrinone would lower PVR in pediatric patients with L-to-R shunts with severe PHT. Methodology : This preliminary, prospective, double-blinded, randomized, placebo-controlled, interventional study includes 24 pediatric patients age 1-15 years old with cardiac L-to-R shunts and severe PHT admitted for cardiac catheterization at a tertiary cardiac referral center. Patients were randomly divided to receive either a single dose of 50 ug/kg IV Milrinone bolus or an equal volume bolus of placebo in a double-blinded fashion to test reversibility of PHT. Outcome variable were measured at the baseline and then 20 minutes after giving the Milrinone or the placebo bolus. For the differences in the values of the outcome variable in the 2 independent groups, Independent t-test & Chi-square test were used. Analysis of Covariance (ANCOVA) was used to control for the influence of a confounding variable, in which the baseline parameters (preintervention – whether placebo or test drug), acted as co-variates. Statistical analyses were done using the SPSS for Windows with level of significance set at p = <0.05. Results : 19 out of 24 patients were included in the study. By paired sample t-test, 3 out of 15 variable in the Milrinone group tested had significant results: SVR (0.023), decrease in mean PAP (0.001), and decrease in PASP (0.006). Using ANCOVA, 8 out of 15 variables tested in the Milrinone group had significant results: Increase in CO by 38.7% (0.046), 333% increase in the PBF (0.046), 10% decrease in PASP (0.002), decrease in diastolic PAP (0.019), decrease in mean PAP (0.010), decrease in Rp (0.013), decrease in Rp:Rs ratio FREE Sheldon Principe Paragas, Teofilo Cantre, Ma. Lourdes SR Casas Department of Pediatric Cardiovascular Medicine, Philippine Heart Center East Avenue, Quezon City PHILIPPINES PAPER FREE (0.044), and decrease in PVR (0.026). There was no significant change noted from the baseline in the placebo group. Conclusion : Milrinone bolus consistently decreases PAP and PVR in pediatric patients with severe PHT secondary to CHD specifically L-to-R shunts. The effect is rapid in onset and well tolerated, with no significant hemodynamic alteration. An intravenous bolus of Milrinone can be used to test for the reversibility of PHT in pediatric patients cardiac L-to-R shunt defects undergoing evaluation for surgical/anatomic correction. No adverse reaction to Milrinone was observed. Keywords : Milrinone, Congenital Heart Disease, Pulmonary Hypertension, Secondary Pulmonary Hypertension, Phosphodiesterase Inhibitors, Pediatrics, Pulmonary Vascular Resistance, FP38 Clinical Course of Children with Congenital Long QT Syndrome at King Chulalongkorn Memorial Hospital PAPER Background: The clinical course of the congenital long QT syndrome (LQTS) appears to depend on the underlying genotype. The prognosis and outcome of these children in Thailand is not known. Objectives: To define clinical presentation, triggering events, response to treatment and prognosis of the congenital long QT syndrome in our institution. Patients and Methods: From August 1998 to July 2006, 10 patients were diagnosed as congenital long QT syndrome by the previously published criteria. Data were collected retrospectively. Baseline clinical information, 12-lead EKG, 24 hours Holter monitoring, exercise stress test, and treatments including response to therapy were determined. Results: Age at presentation varied from in utero (gestational age 32 weeks) to 13.9 (7.5 ± 4.9) years. Five patients (50%) were male. Clinical presentation varied from seizure (30%), syncope (40%), aborted sudden cardiac death (30%), multiform tachyarrhythmia (40%), Torsade de Pointes (10%), palpitation (10%), fetal bradycardia (10%), heart murmur (10%) and asymptomatic family screening (10%). Triggering events were exercise (10%), rest/sleep (30%), both exercise and rest (20%), and unknown triggering events (40%). Other associated diseases were found in 4 patients, including sensorineural hearing loss, LV aneurysm and mental retardation. Twelve leads EKG demonstrated QTc interval varied from 460 to 620 (530 ± 52) milliseconds. Treatments included medication alone (beta-blocker, mexiletine, amiodarone) in 70%, electrophysiologic study and radiofrequency ablation of triggering atrial tachycardia or PVC in 20% and automatic implantable cardioverter defibrillator (AICD) implanted in 2 patients (20%). One patient died from sudden cardiac arrest resulting in brain death. Family history of LQTS was found in 2 patients of the same family. Conclusions: The congenital LQTS at our institution were mostly sporadic cases. Arrhythmic event mostly occurred at rest or during sleep. Except for cases that presented with cardiac arrest, the short term prognosis was good. FREE Monnipa Suesaowalak, Vidhavas La-orkhun, Pornthep Lertsapcharoen, Apichai Khongphatthanayothin *Department of Pediatrics, Faculty of Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand FP39 FREE PAPER INDUCIBILITY OF ATRIAL FLUTTER AFTER FONTAN MODIFICATIONS Bo-Sang Kwon, Eun-Jung Baea, Chung-Il Noha, Jung-Yun Choia, Yong-Soo Yuna, Woong-Han Kimb, Jeong-Ryul Leeb, Yong-Jin Kimb Department of pediatricsa and Department of Thoracic and Cardiovascular Surgeryb, Seoul National University Children’s Hospital, Seoul, South Korea Background: Atrial flutter (AFL) is a common postoperative problem after Fontan repair. The purpose of this study was to evaluate the preventive effect of the elective isthmus cryoablation (EIC) against AFL and identify risk factors for the induciblity of the sustained AFL. Methods: From 2001 to 2006, we performed an electrophysiologic (EP) study upon 59 consecutive patients who had undergone cardiac catheterization after Fontan completion. Results: Mean age was 13.25±5.29 (range: 6-30) years. Follow-up duration was 6.82±3.88 years. In 16/59 (27%) patients, EIC was performed concomitantly with Fontan operations. Sustained AFL was induced in 23 cases and non-sustained AFL in 17. Mean cycle length of AFL was 199.4 ± 47.5 (range: 132-296) msec. Sustained AFL was more likely to occur in patients who were older (16.7±5.2 vs. 11.0±4.1 years, P < 0.001), had a longer follow-up interval (9.2±4.0 vs. 5.3±3.0 years, P <0.001), were older at the time of EP study (13.4±5.0 vs. 8.7±4.0 years, P <0.001) and underwent atriopulmonary connection procedure than extracardiac conduit Fontan procedure (4/5 vs. 2/12, P=0.047). Anatomic diagnoses, perioperative hemodynamics, NYHA functional class, sinus node dysfunction and other previous palliative operations were not associated with an increased incidence of sustained AFL during EP study. There was a high prevalence of the clinical AFL in those patients with an inducible sustained AFL (P=0.063) at intermediate term follow-up. Sustained AFL was less frequently developed in patients who were performed to EIC (1/16 vs. 22/43, P=0.002) (P=0.011, OR 15.7). Multivariate analysis identified chronological age (P=0.022, OR 2.1) and duration of follow-up (P=0.026, OR 1.43) as factors associated with the induction of sustained AFL after Fontan operation. Conclusions: Sustained AFL continues to develop with time after Fontan operation. EIC during Fontan procedure reduced the induction rate of sustained AFL during intermediate term follow up EP study. FP40 LEFT VENTRICULAR MASS IN OBESE CHILDREN PAPER Introduction: Obesity can cause alterations in cardiovascular system even in the absence of systemic hypertension and underlying organic heart disease. Objective: This study was to evaluate the left ventricular mass (LVM) by echocardiography in obese children. Patients & Methods: A cross-sectional study on 49 obese children (age 9.6 ± 2.6 years) underwent 12-lead ECG and echocardiography to assess left ventricular hypertrophy (LVH) and left ventricular mass (LVM), respectively. Results: Mean weight of this cohort study was 61.8 ± 22.0 kg., BMI 29.9 ± 5.8, and percentage of actual weight to ideal body weight for height (%IBW) 172.4 ± 28.3%. There were 3, 18, and 28 children with mild (<140%IBW), moderate (141-160%IBW), and severe obesity (>160%IBW), respectively. Overall, the left ventricular mass index (LVMI) was 38.2 ± 8.9 gm/ m2.7. LVMI in severe obesity (n = 28) was significantly greater than in mild to moderate obesity (n = 2) (40.7 ± 8.8 vs 35.1 ± 8.1 gm/m2.7, p =0.02). Thirty-seven children (75.5%) had normotensive while 12 (24.5%) had systemic hypertension. The LVMI was not significant different in normotensive and hypertensive groups (37.6 ± 9.7vs 40.4 ± 5.7 gm/m2.7). Importantly, there were 13/37(35.1%) in normotensive group and 8/12 (66.7%) in hypertensive group had LVMI >38.6 gm/m2.7 which met the criteria for the LVH. The 12-lead ECG was not the good screening test for LVH. ECG had sensitivity, specificity, positive, and negative predict values in detection of LVH only 28.6%, 78.6%, 50%, and 59.5%, respectively. Conclusions: The left ventricular mass was increased in most of the hypertensive obese children. However, the left ventricular mass in obese children can be increased without associated systemic hypertension. The severity of obese had effect on the increase in left ventricular mass. Weight control to decrease the severity of obesity should be recommended to decrease the left ventricular hypertrophy. FREE Anant Khositseth, Suthutvoravut Umaporn, Chongviriyaphan Nalinee, Ruangkanchanasetr Suwanna Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahdiol University FP41 TRANS-ATRIAL ENDOCARDIAL PACING IN PATIENTS WITH COMPLETE HEART BLOCK FREE PAPER Snehal Kulkarni Aims and Objective: Epicardial lead placement is the standard approach for patients requiring permanent pacemaker following Fontan completion due to restricted venous access. We report two patients with corrected transposition of great vessels, large ventricular septal defect with severe pulmonary stenosis who underwent Fontan completion. They had associated Complete Heart block. They underwent transatrial endocardial lead placement during surgery. Methods : Permanent pacing lead was placed through the atrial appendage and anchored into the endocardium. The other end of the lead was brought out through the pleural space into a subcutaneous pocket in the infraclavicular region, where it was later connected to the pacemaker generator. The sensing threshold obtained were 0.8 and 0.6 volts respectively at the time of implantation. The sensing and pacing characteristics were analyzed 3 monthly up to 1 year. Results : Both the patients had uneventful postoperative course. The pacing and sensing thresholds have remained stable through a follow-up period of 12 and 9 months respectively. Both the patients are on antiplatelet drugs and anticoagulatants. There were no thromboembolic complications. Conclusion: Transatrial endocardial lead through a purse string in the atrial appendage avoids the problem of increasing chronic stimulation thresholds typically observed with epicardial leads. Such an approach would be useful in patients, who have undergone Fontan type procedure resulting in less frequent lead and generator replacements, FP42 Brain natriuretic peptide and cardiac function in doxorubicin administrative cancer children PAPER Background: Doxorubicin-induced cardiomyopathy was the serious complication of cancer therapy in children. Conventional methods to screen early ventricular dysfunction were limited. This study was conducted to use serum NT-pro BNP level to early diagnose ventricular dysfunction in children. Patients and methods: There were 55 cancer patients with accumulative dose of doxorubicin < 300 mg/m 2 (group 1), 49 cases with dose > 300 mg/m2 (group 2) and 52 cases as a control group (group 3). Electrocardiogram, chest roentgenogram, echocardiogram and serum NT- proBNP level were studied. Results: There were significantly higher serum NT-pro BNP level in group 2 than group1 (384±291 VS 92±89; p = 0.001) and than group 3 (79±92; p = 0.001) in patients age 1-10 years. At serum NT-pro BNP level >1SD of control group, there was 3.8 times (95%CI, p= 0.02) to have abnormal >2 parameters of diastolic function. There were 2.8 times (95%CI, p = 0.03) of patients in group 2 to have abnormal 2 parameters of diastolic function and 8 times ( 95%CI, p = 0.001) of chance to have serum NT-pro BNP level >1SD when compare to patients in group 1. Conclusion : There was an association between serum NT-proBNP level >1SD, accumulative doses of doxorubicin > 300 mg/mand early left ventricular diastolic dysfunction (especially E/A <-1 SD and PVA > 1SD). Serum NT-proBNP level >1SD have high propability of early doxorubicin-induced cardiomyopathy in children age 1-10 years. FREE W. Hongkan,1 J. Soongswang,1 G.Veerakul,1 K. Sanpakit,1 K.Punlee,2W. Rochanasiri,2 S.Udompunturak3 1 Department of Pediatrics, Siriraj Hospital, Faculty of Medicine, Mahidol University, 2 Prannok Road, Bangkok, 10700, Thailand 2 Her Majesty Cardiac Center, Siriraj Hospital, Faculty of Medicine, Mahidol University 3 Research Promotion Center, Siriraj Hospital, Faculty of Medicine, Mahidol University FP43 FREE PAPER Comparison of transcatheter closure of atrial septal defect using alternative imaging techniques Kritvikrom Durongpisitkul, M.D., Jarupim Soongswang, M.D., Duangmanee Laohaprasitiporn, M.D., Prakul Chanthong, M.D, Apichart Nana, M.D., Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand Objectives: Transcatheter closure has become a standard procedure for patients with atrial septal defect (ASD). Imaging techniques for sizing the ASD have evolved over past few years. We compare the results of transcatheter closure of ASD using three different imaging methods: 1).Transesophageal echocardiography guide with balloon sizing (TEE & Balloon) under general anesthesia. 2). Transesophageal echocardiography without balloon sizing (TEE) under general anesthesia, and 3). Intracardiac echocardiography (ICE) under local sedation. Patients & Methods: Patients having an ASD and had transcatheter closure between January 1999 to July 2006 were selected. Comparison was made regarding age, ASD size: device size, procedure (proc) and fluoroscopy (flu) time. Results: There were 402 patients who had ASD. The results were shown in table. Parameter Age (yr) Weight (kg) ASD size (mm) Device size (mm) Qp:Qs Device/ASD ratio Proc time (min) Flu time (min) TEE&balloon N = 299 23.4 9.4 46.9 17.4 TEE N = 56 22.9 7.4 50.1 16.7 ICE N = 47 21.6 6.4 55.2 16.3 P=value 0.68 0.007* 23.4 9.4 22.9 7.3 21.6 6.4 0.453 26.7 7.2 4.0 3.1 28.4 7.9 3.2 1.8 27.2 6.3 4.1 3.2 0.304 0.177 1.25 1.23 1.27 0.21 1.31 0.27 0.949 51.7 23.7 44.7 15.5 49.8 21.2 0.085 16.5 9.2 11.6 4.7 9.1 2.6 < 0.01* The patients in the ICE group were older than the other two groups. However, there was no significant difference between ASD size, Qp:Qs, device size, or device:ASD ratio among three groups. Although the average procedure time was not different three groups, however, there was a significant shorter fluoroscopy time in the ICE group. Conclusions: We have demonstrated that Transcatheter closure of ASD using alternative imaging tools such as TEE without balloon sizing or ICE can achieve the same results as conventional methods by using general anesthesia and TEE with balloon sizing. The advantage of alternative imaging methods such as ICE is the shorter fluoroscopy time. . FREE PAPER FP44 FREE PAPER A STAGED CATHETER-SURGERY APPROACH IN TETRALOGY OF FALLOT WITH PULMONARY ATRESIA AND DIMINUTIVE PULMONARY ARTERIES Seong Ho Kim, Kim SJ, Han MY, Kang IS, Bae EJ , Lee HJ, Lee YT Department of Pediatrics , Halla General Hospital Objective: Tetralogy of Fallot with pulmonary atresia and diminutive pulmonary arteries(PAI<90) is difficult to manage, even though several methods of treatment have been tried. A new collaborative approach has been introduced since stenotic pulmonary arteries could be enlarged by balloon dilation. Methods: We catheterize to get detailed pulmonary anatomy and collateral circulation, and establish right ventricle to pulmonary arterial continuity by surgery, and re-catheterize to dilate stenotic pulmonary arteries and to embolize collaterals. Finally, we close ventricular septal defect by surgery. Results: Eighteen patients(PAI=55±18) followed this staged approach. Fourteen of 18 patients(78%) could achieve complete biventricular repair, and 3 patients died - 2 after first surgery, and one after total correction. The final mean pressure ratio of right ventricle to aorta after total correction was 0.63. Conclusions: Establishment of early right ventricle to pulmonary arterial continuity with subsequent pulmonary ballon angioplasty and coil embolization of collaterals allow eventual successful complete repair in 78% of patients. This may be a superior outcome compared with those of previous reports. Nevertheless, long-term follow-up is warranted. FP45 NITINOL VASCULAR PLUG FOR PERCUTANEOUS OCCLUSION OF CORONARY CAMERAL FISTULA - A CASE REPORT FREE Prem Sekar, Pramod Jaiswal Frontier Lifeline, Chennai, India PAPER Catheter based occlusion of coronary fistula is an accepted alternative to standard surgical closure. Commonly used occlusion material are stainless steel coils, Amplatzer PDA occluder and detachable balloons. We report the case of a 6 year old child with a large coronary cameral fistula successfully occluded using a 10mm Amplatzer Nitinol vascular plug. The symptomatic child (NYHA class I) had presented with a continous murmur, a cardiothoracic ratio of 60% on chest X-ray and echocardiographic finding of a large coronary fistula from the left coronary system draining in to the RA. Cardiac catheterization immediately prior to occlusion of the fistula revealed a 2:1 left to right shunt. Post procedure, at three months follow-up, the child reported symptomatic improvement and echocardiography continued to show complete abolition of the left to right shunt. This confirms that the Nitinol vascular plug is an effective device for satisfactory and safe occlusion of moderate to large coronary fistulae. FP46 FREE PAPER ELIMINATING PEDIATRIC CORONARY STENOSIS IS POSSIBLE USING SIROLIMUS-ELUTING STENTS James Yashu Coe, W.J. TYMCHAK1, J. RUTLEDGE1, AND J.R. BURTON1 1 University of Alberta, Edmonton, Alberta Objective: Transluminal coronary angioplasty and stenting are uncommon events in infants and children. Maintaining patency in a growing child may be a concern. Sirolimus-eluting stents have been shown in adult coronary artery disease to have significantly lower incidence of restenosis. Methods: Since May 2002, Sirolimus-eluting stents (n=4) have been used in 3 children, 9 months to 13 years of age, with coronary artery stenosis. Two of the children were moribund at the time of the procedure. The stenoses occurred after surgical repair of anomalous left coronary artery, pulmonary valve implantation and heart transplantation. All procedures were performed under general anesthesia. All patients were treated with heparin and abciximab during the procedure, aspirin and clopidogrel for six months. Results : All procedures were performed under general anesthesia. All patients were treated with heparin and abciximab during the procedure, aspirin and clopidogrel for six months. Sirolimuseluting stents were successfully deployed in all patients without undesirable embolization or vascular injury. Cardiac function was restored in the critically ill patients and discharged from hospital. Recatheterization, up to two years in one, showed patency of the stented vessel and appropriate growth of the adjacent coronary artery. Conclusions : This study showed that it is feasible to treat coronary artery stenosis in the young by balloon angioplasty and maintain their patency using Sirolimus-eluting stents. FP47 TRANSCATHETER PLATINUM-COATED NITINOL OCCLUDING DEVICES: PREVENTION OF NICKEL RELEASE PAPER Background: Recently, Nitinol-based alloys are widely used in medical products, including transcatheter occluding devices in cardiac intervention. However, possibility of systemic nickel release leading to a rise in serum concentrations of nickel after device implantation should be concerned. In this study, the atrial septal defect (ASD) and patent ductus arteriosus (PDA) occluding devices were braided from Nitinol wires and coated with Nanoplatinum. With Nanotechnology, layers of platinum were coated on the surface of meshed Nitinol wires in order to prevent the exposure of Nitinol alloy to the blood after implantation. Objective: Our purpose was to evaluate the change in serum nickel concentrations in the patients before and after transcatheter ASD and PDA closure with platinum-coated Nitinol occluding devices. Method: In 50 patients (25 ASD cases and 25 PDA cases), blood samples were taken during the cardiac catheterization before and at 1, 3, 7, 30, 90 and 180 days after device implantation. Blood samples in 104 normal adult populations were also included. Serum nickel concentrations were measured by atomic absorption spectrophotometry. Results: The mean serum nickel levels were 0.65, 0.63, 0.67, 0.55, 0.53 and 0.42 ng/ml, before and at 1, 7, 30, 90 and 180 days after device implantation in the ASD group; and 0.59, 0.57, 0.56, 0.55, 0.61 and 0.46 ng/ml, before and at 1, 3, 7, 30, 90 and 180 days after device implantation in the PDA group; respectively. The mean level in 104 normal populations was 0.67 ng/ml. There was no statistical difference in serum nickel concentrations before and after device implantation in both the ASD and PDA groups. The serum nickel levels in the 50 patients were not significant different from those in the normal populations. Conclusion: Layers of platinum coated on the surface of meshed Nitinol-wires are able to cover the exposed surface of the Nitinol alloy. Prevention of serum nickel release by using platinumcoated Nitinol occluding devices is possible. FREE Pornthep Lertsapcharoen, Khongphatthanayothin A, La-orkhun V, Supachokchaiwattana P Division of Pediatric Cardiology, Department of Pediatrics Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand FP48 FREE PAPER COMPARISON OF BALLOON ANGIOPLASTY AND PRIMARY STENTING IN CONGENITAL HEART DISEASE Roymanee S, Durongpisitkul K, Soongswang J, Laohaprasitiporn D, Chanthong P, Nana A. Division of Cardiology, Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, 10700. Thailand Objective: To compare the results and complications of balloon angioplasty (BA) with stent implantation (SI) for vascular stenosis in congenital heart disease. Background: Traditionally BA was used primarily for angioplasty. However, due to poor results in some patients, primary stenting was increasingly acceptable for management of resistant lesions. We compared the results of using BA and SI. Methods: A retrospective study of 80 patients who underwent BA or SI between January 1998 and December 2005 at our institution. Procedural success is determined by either increasing of the stenotic diameter > 50% (anatomic success) or decreasing of the pressure gradient > 50% (hemodynamic success). Result: The majority of cases were pulmonary artery stenosis (66.3%) and coarctation of aorta (25.0%). Of these 80 patients, 27 patients underwent SI. The immediate results have shown in Table 1. There was a significant difference in diameter increment percentage and post-dilatation pressure gradient between two groups. Neither mortality nor significant morbidity was observed in each procedure. Table 1. Comparison between the two groups Age (yr) Body weight (kg) Stenosis diameter (mm) Pre-dilatation Post-dilatation % Diameter increment Anatomic success Pressure Gradient (mmHg) Pre-dilatation Post-dilatation1 % Gradient reduction Hemodynamic success BA (Mean + SD) 13.5 16.7 29.8 17.3 SI (Mean + SD) 15.6 11.6 36.1 23.1 P value 6.9 4.2 9.2 5.0 50.0 56.4 15/48 (31.3%) 5.6 2.8 10.0 2.8 114.9 71.2 21/24 (87.5%) 0.205 0.518 < 0.001 25.9 18.1 4.5 16.1 51.2 37.3% 19/35 (54.3%) 25.1 17.9 4.2 5.5 69.4 48.4% 17/19 (89.5%) 0.866 < 0.001 0.131 0.579 0.172 Conclusion: Primary stenting showed a better immediate outcome for achieving larger final diameter and reliving of post-dilation gradient when compared to balloon angioplasty alone. FREE PAPER FP49 FREE PAPER Transcatheter closure of Secundum Atrial Septal Defect in Children Wei Gao, ZHOU Ai-qing , YU Zhi-qing , LI Fen, ZHANG Yu-qi, SUN Kun, ZHONG Yu-min Shanghai Children’s Medical Center Objective : To discuss the indication 0methodology and complication preventing in transcatheter closure of secundum Atrial septal defect(ASD). Background : ASD transcatheter occlusion techniques have become an alternative to surgical procedures. A number of different devices are available for transcatheter ASD closure. The type and rate of complications are different for different devices. Methods : Retrospective analysis was performed on the patients treated from October 1998 to January 2006. Transcatheter closure of secundum ASD with Amplatzer septal occluder (ASO) was performed in 226 cases, of whom 3 mutiple ASD, 3 associated with pulmonary stenosis (PS), 3 patent ductus arteriosus (PDA). Age 0.8~17.0 years (mean 7.5±2.8 years), weight 6.7~88Kg (mean 23.7±7.8 Kg). They are all met with criteria for transcatheter closure received this operation. The balloon-stretched diameter of ASD was determined by fluoroscopy,ultrasound and measuring plate. A choice of device size was identical to or within 2mm larger than the stretched diameter(SD). 6 cases associated with PDA or PS were performed simultaneously PDA closure with device or balloon dilation. Follow up with echocardiography and clinical examination. Results: In 226 cases with transesophageal echocardiography (TEE) or trans-thoracic echocardiography (TTE), ASD mean diameter 12.9±5.6 mm (6.5~34.5 mm), pulmonary mean pressure 29.0 5.0mmHg (25.0~62.0 mmHg), SD 15.7±4.8mm (8.0~38.0 mm). The diameter of these devices were 15.0±5.0mm (8mm-38mm). The devices were successfully implanted in 220 cases. Of whom 3 were multiple ASD with one device occlusion. 6 cases associated with PDA or PS were performed successfully PDA occlusion or balloon dilatation. The immediate, one month and one year complete occlusion rate were 97.7% (215/220), 99.1% (218/220), 99.1% (218/220) respectively. A trivial hemodynamic insignificant residual shunt remained in 2 cases. No complication occurred except transient atrial premature beats and II0I A-V block happened in 5 cases (4.5%)and 1 cases (0.9%). Procedure time ranged from 25~68 minutes and fluoroscopy time from 12~36 minutes .The total follow up was one month to 7.4 years. No unsatisfactory device position or embolization .No clinical evidence of bacterial arteritis. Conclusion Transcatheter closure of secundum ASD using the ASO is a safe and effective alternative to surgical repair. Transcatheter closure of secundum ASD associated with small anterior, inferior or posterior rim is feasible using ASO. Infant and young children can be performed ASO only if the diameter of disk is smaller than the diameter of atrial septum. Multiple ASD is not contraindicaton for intervention. If the diameter of ASD >36mm the device choice was depent on measureing maximum diameter of ASD with Echo. TTE is suitable for small ASD and TEE for bigger ASD. The routine or other advanced methods were performed in using ASO. It is very important to prevent air embolism and atrial perforation. General Cardiology FREE PAPER FP50 FREE PAPER PULSE OXIMETRY SCREENING FOR CRITICAL CONGENITAL HEART DISEASE IN ASYMPTOMATIC NEWBORNS RUANGRITNAMCHAI CHATREE*, PONGPANICH BOONCHOB ** * Department of Pediatrics, Synphaet Hospital, Bangkok, Thailand ** The Cardiac Children Foundation of Thailand Aim: To determine the prevalence of Critical congenital heart disease (CCHD), pulse-oximetric screening combined with clinical examination is superior in the diagnosis of congenital heart disease to clinical examination alone and the effectiveness of a pulse-oximetric screening performed on the first day of life for the detection of CCHD in otherwise healthy newborns Methods: Pulse oximetry was performed on asymptomatic newborns at >24 hours of age. If pulse-oximetric screening for O2saturation was < 95%, echocardiography was performed. Data regarding true and false positives as well as negatives were collected and analyzed. Results: Pulse-oximetric screening was performed on 1609 asymptomatic newborns, and 3 cases of CCHD were detected (1 transposition of the great vessels, 1 complete atrioventricular canal with moderate tricuspid regurgitation and 1 ventricular septal defect with patent foramen ovale with moderate tricuspid regurgitation (TR) with pulmonary regurgitation). The prevalence of CCHD among all live births was 1 in 543 and among the screened population was 1 in 536 (sensitivity: 100%; specificity: 99.8%; positive predictive value: 100%; negative predictive value: 100%; accuracy: 99.8%). Conclusion: Routine newborn auscultation for murmurs is an insensitive method for early detection of critical congenital heart disease, Routine pulse oximetry may improve the early diagnosis. If postductal oxygen saturation is persistently below 95% or if clinical signs occur, referral to a cardiology unit is suggested. Key words: pulse • oximetry • screening • newborn • heart Abbreviations: CHD, Congenital heart disease • CCHD, Critical congenital heart disease FP51 PRENATAL DIAGNOSIS AND OUTCOME OF FETUSES WITH TETRALOGY OF FALLOT PAPER Objective: To review the correlation of the prenatal echocardiographic(echo) features and postnatal outcome in fetuses with tetralogy of Fallot(TOF). Materials and Methods: Between January 2000 and July 2006, a total of 78 fetuses were diagnosed with TOF in our institution. Among these, 43 fetuses had complete prenatal and postnatal echo data and were subject of this study. During fetal echo we measured diameter of all valves, great arteries and chambers and also flow velocity across great arteries as well as complete cardiac and non-cardiac structures. Results: Among 43 fetuses, 40 had a pulmonary valvar diameter below normal range(±2SD) at some point during gestation. The ratio of the diameter of the aortic to the pulmonary valve was upper than normal in all fetuses. The pulmonary arterial (PA) Doppler velocity was higher than normal in all fetuses. There was a significant increase in PA Doppler velocity from fetal to postnatal life. Mean PA Doppler velocity was 1.17±0.27 m/s at 2nd trimester, 1.36±0.42 m/s at 3rd trimester, 2.34±0.55 m/s at birth, and 4.29±0.44 m/s at 3month after birth. Location of VSD was perimembranous in 34, subarterial in 6, muscular outlet in 2 and total conal defect in 1. Among 43 newborns, 21 underwent one stage total correction at 10.4±4.0 months, 6 underwent systemicpulmonary shunt operation at 2.6±2.9 months and 16 are awaiting operation. There was no significant difference in echo measurements between fetuses who subsequently underwent onestage total correction and fetuses who underwent shunt operation. Conclusions: In TOF, pulmonary valve hypoplasia and increased pulmonary arterial Doppler velocity are detected from fetal life. Progression of obstruction in right ventricular outflow tract was observed during fetal life and after birth. There was no prenatal echo feature that can predict the need for early intervention. FREE Jhang W.K, 1 Won H.S, 2 Kim Y.H,1 Ko J.K,1 Jung S.H,3 Park J.J,3 Yoon T.J,3 Seo D.M,3 Park I.S1 Department of Pediatrics, 1 Department of Obstetrics, 2 Department of Cardiac Surgery3 University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea FP52 FREE PAPER ADOLESCENCE AND ASSIST DEVICES, ARE THEY A GOOD MATCH? Lisa Underwood c- Ward 23b, Starship Children’s Hospital, Private Bag 92-024, Auckland 1023, New Zealand This presentation will delve into the experience of a teenage boy who was one of the first recipients of the newly developed left ventricular assist device. The PCCS in Auckland, New Zealand, although a small isolated country provides world class treatment using the latest technological developments available on the world stage. This young person had a long history of dilated cardiomyopathy, but in his early teens his function had decreased to the point of making him dependent upon hospital treatments. When his function decreased to a critical point a decision was made to utilize the Melbourne team, in Australia, to implant the LVAD. He was transplanted 10 months later. This case study will delve into the heart and soul of life as a chronically ill teenager and his family, the challenges faced and overcome, with the support of a variety of networks within the hospital. By Lisa Underwood, c- Ward 23b, Starship Children’s Hospital, Private Bag 92-024, Auckland 1023, New Zealand. FP53 QUANTIFICATION OF AORTOPULMONARY COLLATERAL BLOOD FLOW AFTER BIDIRECTIONAL GLENN PROCEDURE PAPER Background: Hemodynamics of bidirectional Glenn (BDG) circulation with aortopulmonary collateral arteries are not well understood because of difficulty in quantifying the collateral blood flow. This study attempted to provide a new method to quantify the aortopulmonary collateral blood flow after BDG procedure. Methods: Five patients after BDG procedure underwent cardiac catheterization and nuclear scintigraphy using 99m-Tc labeled macroaggregated albumin (MAA) administrated from lower extremity vein. Albumin aggregates were distributed to lung and body through collateral arteries and systemic arteries, respectively. The collateral/systemic flow ratio (=M) was measured from total body image: M = total lung count/ (total body count - total lung count). Combining Fick principle and the MAA-based collateral/systemic flow ratio, collateral/pulmonary flow ratio (Qc/ Qp) was obtained, where pulmonary flow = collateral flow + superior vena cava flow: Qc/Qp = (pulmonary venous saturation - inferior vena cava saturation)/(1+1/M)(systemic saturation - Inferior vena cava saturation). Using this formula, we calculated the collateral blood flow. Results: The median age at operation was 1.52 years (range, 1.0 to 2.0), and the median age at catheterization was 2.61 years (range, 1.83 to 9.47). The collateral/systemic flow ratio was 0.48 ± 0.12 (mean ± SD), and the collateral/pulmonary flow ratio was 0.60 ± 0.17. The collateral blood flow was 1.67 ± 0.30 L/min/m2 (range, 1.34 to 2.01). Pulmonary vascular resistance (PVR) was 1.97 ± 0.79 Woods units m2. If we ignore the collateral blood flow (assuming M = 0), calculated PVR would be 3.08 ± 1.30 Woods units m2, overestimated by 31 to 64%. Conclusions: The aortopulmonary collateral blood flow after BDG procedure can be calculated with new MAA-catheterization-combined approach. The collateral blood flow plays an important role in BDG circulation. This approach enables us to avoid overestimation of PVR in considering the indication of Fontan procedure. FREE Ryo Inuzuka, Hiromichi Nakajima, Kouta Agematsu, Yuji Naitou, Kenji Sugamoto, Shunsuke Tatebe, Mitsuru Aoki, Tadashi Fujiwara, Hiroyuki Aotsuka The Divisions of Pediatric Cardiology and Pediatric Cardiothorascic Surgery, Chiba children’s hospital FP54 FREE PAPER Acute Cardiac Remodelling After Transcatheter Closure of Atrial Septal Defect in Adult Patients En-Ting Wu, Teiji Akagi, MD, PhD, Tsutomu Taniguchi, MD, PhD , Tsumoru Maruo, MD, PhD , Shinichi Otsuki, MD, PhD, Shunji Sano, MD, PhD Cardiac Care Center, Okayama University Hospital Objectives: To evaluate the acute cardiac remodeling after transcatheter closure of ASD in adult patients. Background: Transcatheter closure of atrial septal defect (ASD) has showed good short-term, mid-term and even long-term results, especially in pediatric patients. But in adults, due to longstanding RV volume overload and subsequent change in pulmonary vasculature, longer period of cardiac adaptation may be expected after the procedure. There are limited reports about this remodeling in adult patients. Methods: We enrolled 17 adults (mean age 58.4 ±17.3 years old) who underwent successful catheter closure of ASD since August 2005 to July 2006. We arranged routine transthoracic echocardiographic study including LV and RV Tei indexes and serum BNP sampling before ASD closure, one day, 1 month and 3 months after closure. Thereafter, a statistically analysis was performed. Results: We found 1) LVEDD increased, and RVEDD decreased markedly early after the closure of ASD (p <0.05); 2) Tei index of LV declined soon after the procedure, but which of RV showed a trend of increasing level until 1 month, then decreasing at 3 month visit; and 3) BNP elevated one day after closing ASD and declined at the one-month visit. This trend is possibly related to the change of LVEDD. Conclusion: We confirmed that the acute “shrinkage” of the right ventricle and “expansion “of the left ventricle occurred soon after the procedure, even in elder patients. Besides, the LV Tei index decreased after the procedure which indicated the recovery of LV from the compression. The RV Tei index increased initially after the procedure but declined one month after. The elevation of plasma BNP was related to the acute extension of LV chamber but it relationship with the LV function remained unclear. FP55 DOES THORACIC SITUS ACCURATELY PREDICT ATRIAL SITUS IN HETEROTAXY SYNDROMES? Symmetrical R lungs Symmetrical lengths L/R ratio < 1.5 Cartilage rings (L) Bilat eparterial Br P value <0.0001 <0.0001 <0.0001 0.4 <0.0001 AUC 0.59 0.69 0.83 0.56 0.81 Sensitivity 0.25 0.41 0.79 0.84 0.66 Specificity 0.94 0.97 0.86 0.29 0.97 Conclusion: In a patient with congenital cardiac disease, the first step in the diagnosis of the segmental combinations is to establish atrial situs. If bronchial situs or symmetry can be identified, this may provide a clue to the heterotaxy syndrome usually associated with severe complex cardiac lesions. In the splenic syndromes, the relationship of bronchi to pulmonary arteries was the best predictor but exceptions do occur. PAPER Background: Symmetry of thoracic viscera and atrial appendages is well known in heterotaxy syndromes. This study was undertaken to assess the reliability of thoracic situs to indicate atrial situs. Method: Thoracic situs was assessed in 247 post mortem case with situs solitus of the atria and 58 cases with heterotaxy (32 with asplenia, 11 polysplenia and 15 others). Five features to assess thoracic situs are lung lobation, the lengths of the right(R) and left (L) main bronchi (Br), the ratio of L/R lengths, the number of cartilage rings, and the relationship of bronchi to the pulmonary arteries. Results: In cases with heterotaxy, symmetry (or isomerism) was found of lung lobation in 42/58 (72%), of bronchial lengths in 78 %, in relationship of bronchi to pulmonary arteries in 91%, and in cartilage rings in 30 %. The L/R ratio was < 1.5 in 83 %. All patients with asplenia had bilateral right atrial appendages. In polysplenia the atrial appendages were morphologically left in 82 %. Predictors for Heterotaxy: FREE LOUISE CALDER Green Lane Paediatric Cardiac and Congenital Services, Auckland, 1030, New Zealand FP56 FREE PAPER Contrast-Enhanced Magnetic Resonance Angiography of Persistent Fifth Aortic Arch In Children Yumin Zhong, Richard B. Jaffe, Ming Zhu, Aimin Sun, Yuhua Li, Wei Gao Shanghai Children’s Medical Center Background: Cineangiography and echocardiography have been utilized to diagnose congenital aortic arch anomalies. However, the visualization of great vessels by echocardiography is limited, while cineangiography requires cardiac catheterization with ionizing radiation. Contrast-enhanced magnetic resonance angiography (MRA) is a noninvasive modality for visualization of congenital aortic arch anomalies. Objective: To evaluate the utility of contrast-enhanced MRA in the diagnosis of persistent fifth aortic arch, a rare congenital aortic arch anomaly, and to compare its diagnostic accuracy with that of cineangiography and echocardiography. Materials and Methods: In four pediatric patients, contrast-enhanced MRA studies were performed for diagnosing persistent fifth aortic arch. The findings of MRA were compared with echocardiographic findings and confirmed by cineangiography and operation. Results: Transthoracic surface echocardiography noted an aberrant vessel arising from the ascending aorta in two of four patients and the etiology of this vessel was uncertain. In the other two patients a diagnosis of coarctation was made. In four patients, only one patient was diagnosed with interruption of the aortic arch. Contrast-enhanced MRA clarified uncertain echocardiographic findings correctly diagnosing persistent fifth aortic arch with fourth aortic arch interruption in all four patients. Conclusion: Contrast-enhanced MRA is a safe, accurate fast-imaging technique for the evaluation of persistent fifth aortic arch, and may obviate the need for conventional cineangiography. Cardiac catheterization may be reserved for some complicated congenital heart disease and for obtaining hemodynamic information. Keywords: contrast-enhanced MR angiography, echocardiography, persistent fifth aortic arch, congenital anomaly, aortic arch anomaly, interruption of the aortic arch YIA1 Transxiphoid approach for children with atrial septal defects Shin Takabayashi 1, Shimpo 1, Kazuto Yokoyama 1, Hiroyuki Ohashi 2, Yoshihide Mitani 2 1 Departments of Thoracic and Cardiovascular Surgery and 2Pediatrics, Mie University Graduate School of Medicine PAPER FREE Objective: Minimal invasive procedures have recently been sought in cardiovascular surgery. We sought to confirm the efficacy of a transxiphoid approach that negates a full sternotomy. Methods: Between January 1999 and June 2005, 29 children underwent surgical direct closure of the atrial septal defect through a transxiphoid approach. Before August 2003, 19 patients underwent the operation, group E, with the remainder, 10, in group L. Mean age and body weight were; group E, 64±30 months and 18±6.2 kg; group L, 71±12 months and 17±1.7 kg. Results: Operation time (127±24 minutes versus 249±35 minutes), cardiopulmonary bypass time (41 8.4 minutes versus 74±13 minutes), and aorta clamp time (11±3.3 minutes versus 23±9.8 minutes) were all shorter in group L than in group E, respectively (p<0.05). Sedative frequency after operation in group L was less than in group E (1.3±1.3 times versus 4.0±3.4 times, p=0.004) and chest drainage duration was shorter in group L than in group E (1.0±0 day versus 1.6±0.7 days, p<0.001). Although water balance of operative day in group L was less than in group E (-284±156 ml versus -138±187 ml, p=0.04), oral intake of operative day in group L was more than in group E (182±92 ml versus 103±61 ml, p=0.04). Group L started walking earlier than group E (post operative day 1.7±0.9 versus 2.7±1.1, p=0.03). Conclusion: It is possible to perform the operation in a short time through a transxiphoid approach. Shortening operation and CPB times leads to the earlier recovery of patients after their operation. YIA2 FREE PAPER Pre-operative Evaluation with Magnetic Resonance Imaging in Tetralogy of Fallot and Pulmonary Atresia with Ventricular Septal Defect Monnipa Suesaowalak, Kritvikrom Durongpisitkul, Prakul Chanthong, Jarupim Soongswang, Duangmanee Laohaprasitiporn, Apichart Nana *Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Bangkok, Thailand Background: Pre-operative evaluation of patients undergone repair of Tetralogy of Fallot and Pulmonary Atresia / Ventricular Septal Defect were generally done by echocardiogram and cardiac catheterization. Our objectives of this study were to compare the findings of Gadolinium (Gd) enhanced cardiac MRI with cardiac catheterization and surgical findings for patients undergone surgical repair of Tetralogy of Fallot and Pulmonary Atresia / Ventricular Septal Defect. Patients and Methods: Patients who underwent repair of Tetralogy of Fallot and Pulmonary Atresia / VSD were prospectively evaluated using cardiac catheterization and cardiac MRI within 3 months. Branch of pulmonary arteries were divided into: main pulmonary artery (MPA), right and left branch pulmonary artery (RPA & LPA), major aortopulmonary collateral arteries (MAPCAs) and minor APCAs. The study results were interpreted blindly. The agreement of findings were compared using Kappa statistics. Surgical findings were compared with cardiac catheterization and cardiac MRI using percentage of patients. Results: There were 43 patients who received both cardiac catheterization and cardiac MRI within 3 months period. The average age was 13.8±8.4 (2-30) years old. There were an agreement among measurement of both main and branches (right and left) pulmonary artery with Kappa statistics more than 0.8. MRI was able to identify more branches of MAPCAs compared to cardiac catheterization. There were 15 patients who underwent surgical repair. There were an agreement of main and branches (right and left) pulmonary artery 78% - 85%. MRI can identified more branches of MAPCAs compare to surgical findings. Conclusions: The results of this study indicate that Gd-enhanced 3D MRA is a feasible, fast and accurate technique for identification of all sources of pulmonary blood supply in patients with Tetralogy of Fallot and complex Pulmonary Atresia. The study was a noninvasive alternative to cardiac catheterization. MRI can better delineated small (minor) branches of collateral arteries that were used as a guide for planning of transcatheter coil embolization in addition to routine cardiac catheterization. YIA3 MANAGEMENT AND OUTCOME OF FETAL ATRIAL FLUTTER IN THE KOREAN POPULATION PAPER Introduction: The aim of this study was to evaluate fetal atrial flutter(AF) and the efficacy of maternally administered antiarrhythmic agents, postpartum management and outcome. Methods: Fetal AF cases seen in three institutions between June 1997 and June 2006 were reviewed retrospectively. Results: AF was diagnosed prenatally in 12 fetuses. Mean age at detection was 35.3+/4 weeks’ gestation. Most fetuses with AF had structurally normal hearts except one with critical PS. AF was incessant in 9 and intermittent in 3, with a mean atrial rate of 422+/-103 beats/min and a mean ventricular rate of 216+/-37 beats/min. A predominance of 2:1 atrioventricular (AV) conduction was observed except one 1:1 AV conduction. In 2 of 12 cases supraventricular tachycardia coexisted with AF. Fetal hydrops was seen in 3 patients and were treated in utero with maternal therapy, 1 received digoxin and 2 mutidrug therapy (digoxin and flecainide). In the nonhydropic group of 9 patients, one case of fetal AF resolved spontaneously in utero and one case treated in utero with digoxin and another case received multiple drugs, digoxin and flecainide with atrial ectopic tachycardia after birth. And the remaining 6 were delivered immediately. Three fetuses received no medication and were delivered and AF subsided. Three neonates were in AF at birth. Rhythm control could be achieved in 2 patients with propafenone (n = 1), or electrical cardioversion (n = 1) without any relapse. But one patient with 2:1 AV conduction died after birth due to complication from renal and heart failure in spite of multiple therapy without conversion to sinus rhythm. Recurrence of AF after initial conversion to sinus rhythm did not occur in any of the patients. Conclusion: Even though fetal AF can be a serious and life-threatening rhythm disorder, therapy can be useful for most of them. And once fetuses with AF survive, their longterm prognosis seems to be excellent. And it appears that recurrence of AF is unlikely beyond the neonatal period. FREE Song Min Seob 1,Won HS2, Jeong DH3, Hong YM4, Park IS5 Department of Pediatrics1 and Obstetrics and Gynecology3, Paik Hospital, Inje University,Pusan,Department of Obstetrics and Gynecology2 and Division of Pediatric Cardiology5, Asan Medical Center, Ulsan University, Seoul, Department of Pediatrics4, DongDaeMun Hospital, Ewha Woman’s University, Seoul, Republic of Korea YIA4 FREE PAPER Regression of Dilated Cardiomyopathy after Switching Iron Chelator from Deferoxamine (DFX) to Deferiprone (L1) Tzu-Yao Chuang, Jeng-Sheng Chang, Yu-Chih Huang, Kang-Hsi Wu, Ching-Tien Peng Dep of Pediatrics, Devision of Cardiology, China Medical University Hospital , Taichung, 404, Taiwan Background: Cardiac dysfunction from iron deposition is the most common cause of mortality in patients of thalassemia major after long-term blood transfusions. Deferoxamine (DFX) subcutaneous or intravenous iron chelator, though effective, has long been complained of user inconvenience and poor compliance. With introducing of a new oral iron chelator, Deferiprone (L1), in latest years, the medication compliance improves substantially. This study tries to compare the cardiac protection effects between DFX and L1. Method: From Nov. 1989 through Jan. 2005, nine thalassemia major patients receiving deferoxamine (DFX) injection therapy for more than 10 years but complicated with some degree of myocardial dysfunction were enrolled into the study to switch from DFX to L1, at a dose of 75 mg/kg/day. Echocardiographical studies focusing on systolic, diastolic and global functions of the left heart were performed regularly every 6 months. Mean values of each parameter are compared between the DFX and L1 periods by paired t-test and Wilcoxon signed-rank test. Result: Significant improvements of global cardiac function are shown in reducing of E-point to Septal separation (EPSS) (8.11+ 3.67 vs. 2.9+ 0.89 mm, P<0.01), EPSS/LVDd (left ventricle diastolic dimension) ratio (0.19 + 0.08 vs. 0.06 + 0.02, P< 0.01) and left atrium/aorta (LA/AO) ratio (1.27+ 0.15 vs. 1.10+ 0.13, P= 0.02). Conclusion: The myocardial dysfunction of -Thalassemia can be reversed by regular oral iron chelator L1. YIA5 THE USE OF AEROSOLIZED PROSTACYCLIN ANALOGUE (ILOPROST) IN THE PREVENTION OF POSTOPERATIVE PULMONARY HYPERTENSIVE CRISIS AMONG CHILDREN WITH LEFT-TO-RIGHT SHUNTS AND MODERATE TO SEVERE PULMONARY HYPERTENSION PAPER Background: The natural history of an uncorrected congenital heart disease is the development of pulmonary hypertension. This study was conceptualized to determine the effect of aerosolized Iloprost in the prevention of pulmonary hypertensive crisis when given at least 48 hours in the postoperative period. Method: This is a randomized placebo controlled study. The study included pediatric patients (0-19 years old) with congenital heart disease: left to right shunts with moderate to severe pulmonary arterial hypertension admitted at the Philippine Heart Center from March 2005January 2006. PA pressure was determined by echocardiography using pulmonary acceleration time. Patients with PA pressure of >60 mmHg were included in the study. Patients were divided into two groups: Group I received every 6 hours aerosolized Iloprost for 48 hours, and group II was the control group, given nebulized saline solution. Randomization was done by using a table of random samples. Evaluation of parameters, including Doppler echocardiogram for PAT was done at 0-hour, 24-hour, and 48-hour postoperatively. There were 19 patients enrolled in the study: ten in Group I and nine in Group II. Aerosolized Iloprost was given at a dose of 25 ng. kg-1 min-1, given every 6-hour interval for 48 hours. Results: Of the nineteen patients, 58% were males with a 1.4:1 male to female ratio. Mean age was 7.25 + 5.93 in the Iloprost group and 3.63 + 5.19 in the placebo group. Both were comparable in their preoperative baseline characteristics, with a mean PAP of 60.37 + 12.66 and 68.55 + 10.70 respectively (p <0.05). Ventricular Septal Defect was the most common lesion (59%). Iloprost nebulization in Group I showed statistically significant increase in oxygen saturation every 6-hour nebulization till the 48th hr (p=0.05). No significant change in systolic and diastolic BP was seen at 0-hr, 6 and 24th hr. Pulmonary to systemic flow ratio increased significantly compared with the placebo at 0-hour, 24-hour and 48-hour (p<0.05). Pulmonary resistance significantly dropped by 9 secs/min (p0.012) but this was not significantly different from group I. Drop in PAP until the 48th hour was not significant. Increasing the sample size is recommended. There was no mortality in both groups with no episode of desaturation despite an increased PAP. Conclusion: Aerosolized Iloprost given every 6-hour is an important pharmacologic agent to be used in the immediate post-operative period to prevent pulmonary hypertensive crisis. FREE RUZENETTE FELICITAS HERNANDEZ, J., FERRERA BT., LAZARO E., LAGAMAYO M., CASAS M. Philippine Heart Center, East Avenue, Quezon City, Philippines YIA6 FREE PAPER LEFT PULMONARY ARTERIOPLASTY -SLIDING TECHNIQUE AND MAIN PULMONARY ARTERIAL TRANSLOCATION TECHNIQUE Masahiro Yoshida, Yoshihiro Oshima, Chikashi Shimazu, Hironori Matsuhisa, Tomonori Higuma, Hiroaki Takahashi The Department of Cardiovascular Surgery, Kobe Children’s Hospital Surgical arterioplasty for left pulmonary branch stenosis has often produced unsatisfactory results. We report new operative techniques involving sliding technique, a modification of end-to-end anastomosis, and main pulmonary arterial translocation technique. These techniques provided widely patent and non-redundant anastomosis. (Techniques) The operation is performed through a median sternotomy. In cases that require an additional aortopulmonary shunt, a standard left posterolateral thoracotomy is performed. The left pulmonary artery and main pulmonary artery are completely dissected to allow anastomosis without tension, and the stenotic portion is transected. For sliding technique, the distal incision is made on the superior aspect of the left pulmonary artery and the proximal incision is made on the inferior aspect of the main pulmonary artery. The anastomosis is completed with running suture of 7-0 polypropylene. If neccesssary, autologous pericardium patch is used at the distal left pulmonary artery. For the other technique, the main pulmonary artery is transected and the stump is translocated to the distal left pulmonary artery and anastomsis is completed. (Patients and results) From 2002 to May 2006, 11 patients, median age was 1.5 years, underwent the left pulomnary arterioplasty using sliding techniques or translocation technique. The sliding technique was performed in eight of them at the time of biventricular repair in six, bidirectional Glenn anastomosis in one and modified BT shunt in one. In five of them, autologous pericardial patch was used to avoid tension. The other two patients underwent translocation technique at the time of bidirectional Glenn anastomosis in one and modified BT shunt in one. Postoperative angiograms or three-dimensional computed tomography showed patency of left pulmonary artery with enough size of diameter in all patients. (Conclusion) These two techniques are useful for patients with severe left pulomonary stenosis by means of providing enough continuity of autologous pulmonary tissue.