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division of Pediatric Cardiology Mission The mission of the Division of Pediatric Cardiology is: •To provide comprehensive clinical services from the fetus to children and adults with congenital heart disease as well as to children with acquired heart disease 221 •To train the next generation of clinical pediatric cardiologists and physicianscientists through a comprehensive fellowship program of superior quality •To perform high-quality clinical and basic research in the fields of heart disease and development •To teach the fundamentals of pediatric cardiology to medical students, pediatric residents, and pediatric and adult cardiology fellows. DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT division of Pediatric Cardiology division of Pediatric Cardiology faculty and staff Vivek Allada, MD Evonne Krushansky, DO Jamie Bloch, BSN, MSN, CRNP Professor of Pediatrics Interim Chief, Division of Pediatric Cardiology Clinical Director, Pediatric Cardiology Codirector, Heart Institute Assistant Professor of Pediatrics Cardiac Catheterization Laboratories Elisa Marcuccio, MD Janet Kulka, MS, PA-C Assistant Professor of Pediatrics Inpatient Services Francis M. McCaffrey, MD Beth Kus, MPAS, PA-C Cardiac Transplant Program Gaurav Arora, MD Associate Professor of Pediatrics Director, Exercise Laboratory Assistant Professor of Pediatrics Associate Director, Electrophysiology Susan A. Miller, MD Concetta Lombardo, BSN, MPAS, PA-C Assistant Professor of Pediatrics Outpatient Services Sara Trucco, MD Assistant Professor of Pediatrics Elena Flores-Noel, DNP, FNP, CRNP-BC Linda M. Russo, MD Outpatient Services, Exercise Stress Lab Lee B. Beerman, MD 222 Professor of Pediatrics Director, Electrophysiology Constantinos Chrysostomou, MD Assistant Professor of Pediatrics and Critical Care Medicine Stephen C. Cook, MD Visiting Associate Professor of Pediatrics and Internal Medicine Director, Adolescent and Young Adult Congenital Heart Disease Center Mark DeBrunner, MD Assistant Professor of Pediatrics Director, Pediatric Cardiology Fellowship Program Stacey Drant, MD Associate Professor of Pediatrics Director, Noninvasive Imaging Johanna L. Drickman, MD Assistant Professor of Pediatrics Brian D. Feingold, MD, MS Assistant Professor of Pediatrics Director, Heart and Heart-lung Transplantation Lizabeth Lanford, MD Clinical Assistant Professor of Pediatrics Jacqueline Kreutzer, MD Associate Professor of Pediatrics Director, Cardiac Catheterization DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT T OVERVIEW OF DIVISION Assistant Professor of Pediatrics Frederick S. Sherman, MD, MBA Professor of Pediatrics, Obstetrics, Gynecology, and Reproductive Sciences Director, Perinatal Cardiology he Division of Pediatric Cardiology forms an integral part of the Heart Institute service line at Children’s Hospital of Pittsburgh of UPMC and is the leading provider of comprehensive pediatric cardiovascular services in Western Pennsylvania and the tristate area. It is an internationally recognized center of excellence in pediatric heart failure, cardiomyopathy, mechanical circulatory support, and heart and lung transplantation. The Heart Institute integrates the various divisions and programs necessary to provide state-of-the-art cardiovascular services to children and young adults with congenital heart disease and to children with acquired heart disease. The components of the Heart Institute include the Division of Pediatric Cardiology (Interim Chief Vivek Allada), the Division of Cardiothoracic Surgery (Chief Victor Morell), the Cardiac Intensive Care Unit (CICU) (Chief Ricardo Munoz), and Cardiac Anesthesia (Chief Steven Lichtenstein). The CICU is a 12-bed unit and is the only dedicated pediatric CICU in the tristate area. The Heart Institute actively participates in numerous National Institutes of Health (NIH)-, foundation-, and industrysponsored clinical and basic research programs. The program has an active pediatric cardiology fellowship program accredited by the Accreditation Council for Graduate Medical Education (ACGME). The CICU also offers postgraduate training in pediatric cardiac intensive care to fellows who have completed training in pediatric cardiology or pediatric intensive care. Achievements for the past year include continued expansion of cardiology services (regional, national, and international; see below), expanded NIH funding to perform multicenter trials in pediatric heart transplantation, continued development of novel mechanical circulatory support devices for use in children, and expansion of the fellowship program. Strong research collaborations exist with Pitt’s Swanson School of Engineering Department of Biomedical Engineering, Thomas E. Starzl Transplantation Institute (STI), and the cardiac genetics research program of Pitt’s School of Medicine’s Department of Developmental Biology under the chairmanship of Cecilia Lo, PhD. 223 T Clinical activities he Division of Pediatric Cardiology provides comprehensive clinical services to children and adults with congenital heart disease as well as to children with acquired heart disease. This clinical program includes ambulatory diagnostic services at 15 locations: the Children’s Hospital of Pittsburgh of UPMC campus in Pittsburgh’s Lawrenceville neighborhood; the three satellite facilities in Wexford, Monroeville, and Bethel Park; Magee-Womens Hospital of UPMC; UPMC Presbyterian; and nine other outreach locations throughout western and central Pennsylvania and the panhandle of West Virginia. With this extensive outreach program, cardiology is taking its services out into the community and has the most extensive outreach services of any program within Children’s Hospital. This year, office visits exceeded 13,000, and half of the patients were seen at locations other than the Lawrenceville campus. The division reached a milestone: more than 2,000 patients were seen in remote outreach locations beyond the metropolitan Pittsburgh area. This model is now being pursued by other divisions. Ambulatory services include diagnostic outpatient office visits, noninvasive imaging including echocardiography (surpassing 16,000 studies for the first time), arrhythmia detection including electrocardiography, 24-hour Holter and 30-day event recorder monitoring, cardiopulmonary exercise testing, and preventive cardiology services. The program supports a comprehensive Perinatal Cardiology Program in collaboration with the obstetrics team at Magee. This program has three facets: neonatal cardiology, fetal cardiology, and maternal cardiology. It is a unique program, and the fetal clinical program is now one of the busiest in North America, eclipsing 2,000 fetal echocardiograms for the first time in its history. The program now performs fetal interventions, notably in utero dilation of the aortic valve to prevent progression of critical aortic stenosis to hypoplastic left heart syndrome. Pediatric Cardiology also provides inpatient and same-day services at Children’s Hospital, and these services include diagnostic and state-of-the-art interventional cardiac catheterization; diagnostic and therapeutic arrhythmia management, including arrhythmia ablation using radiofrequency or cryoablation; and medical management of cardiac dysrhythmias, inflammatory diseases of the heart, and heart failure. The cardiology faculty also follows all children receiving surgical management of congenital or acquired cardiovascular malformations. The pediatric CICU is dedicated to the medical and surgical management of critically ill patients with congenital and acquired cardiovascular disease. This is the only such unit in our region. The improved medical and surgical outcomes of children with congenital heart disease have led to a growing number of survivors flourishing in the community. It is now estimated that there are more adults living with congenital heart disease than children, and adult congenital heart disease comprises approximately 10%–15% of the work of the Heart Institute at this time and continues to grow. The pediatric cardiology division has a formal Adolescent and Young Adult Congenital Heart Disease Service under DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT division of Pediatric Cardiology division of Pediatric Cardiology the direction of Stephen Cook. This program serves to improve and expand the care offered to the growing population of adults with congenital heart disease. As part of a comprehensive program, Cook also directs a Congenital Heart Disease Cardiac Magnetic Resonance Imaging Program at UPMC Presbyterian. The interventional catheterization team continues to perform transcatheter replacement of the pulmonary valve, thus avoiding open heart surgery for selected patients. In fact, Children’s Hospital was the first center to implant such a device in the United States after the Melody Valve was approved for use by the U.S. Food and Drug Administration. Finally, a new initiative in pediatric cardiology this year is a state-of-the art telemedicine echocardiography service that supports the reading of fetal and pediatric transthoracic echocardiograms throughout western Pennsylvania and West Virginia. Cardiology at the Children’s Hospital has always been recognized for its excellence in clinical care, service delivery, and communication with referring physicians. The division has continued to focus on these areas throughout the course of the year. Cardiology faculty routinely receives the highest levels of patient satisfaction from both Children’s Hospital and national surveys. 224 The clinical activities of the division continue to grow, as demonstrated by the spectacular seven-year trends shown in the table below. KEY INDICATORS FY 05 FY 06 FY 07 FY 08 FY 09 FY 10 Outpatient Visits (Total) Outreach outpatient visits Echocardiograms (Total) FY 11 FY 12 % CHANGE 2005–12 8,439 9,43110,95011,76012,27012,75913,00113,629 776 891 908 1,409 1,867 1,947 1,951 2,104 62% 171% 9,69811,12312,89813,27413,96714,74715,22716,425 69% Echocardiograms (postnatal)7,990 9,45211,19411,43512,12612,95813,34714,342 79% Fetal/neonatal echo at Magee-Womens Hospital Vivek Allada, MD RESEARCH Vivek Allada led a study characterizing physician productivity at academic pediatric cardiology echocardiography laboratories. This survey, the first of its kind in this group, aims to understand the practice patterns and manpower needs in pediatric cardiology. Noninvasive assessment of ventricular function, including following heart transplantation: Use the Myocardial Performance Index Derived by Echocardiography to Identify Rejection in Patients after Heart Transplant ADVISORY COMMITTEE MEMBERSHIPS • Cochair, Committee on Echocardiography Lab Organization and Productivity, American Society of Echocardiography Editorships • Guest editorial reviewer, American Journal of Cardiology • Guest editorial reviewer, Congenital Heart Disease • Guest editorial reviewer, Pediatric Research 1,7081,6711,7041,8391,8411,7891,880 2083 22% Holter monitors 493 378 7821,4871,2771,2071,335 1769 259% Exercise stress tests 217295298333358638684763 252% Cardiac catheterization 52252463564164870070874342% Electrophysiology ablations 296066 10886918899 241% Gaurav Arora, MD Research Gaurav Arora’s research interests include management of arrhythmias. T RESEARCH AND OTHER SCHOLARLY ACTIVITIES he Division of Pediatric Cardiology and Heart Center faculty lead multiple active clinical, translational, and basic research programs in cardiovascular disease. The clinical research program is multifaceted, with clinical studies and trials in all aspects of pediatric cardiology, cardiac intensive care, and congenital heart surgery. Particular strengths include the research programs focusing on optimizing outcomes following pediatric heart and lung transplantation and the multidisciplinary program developing novel mechanical circulatory support devices for children with failing hearts. Cardiac genetics research is being carried out in collaboration with Cecilia Lo, PhD, chair of the Department of Developmental Biology within the University of Pittsburgh School of Medicine. Lo is an internationally recognized expert on the genetics of congenital cardiovascular malformations, working with mouse models and human subjects. Finally, the research arm of the cardiology division has completed development of a comprehensive cardiology database, based on a long-standing database carrying patients from the 1960s to the present and incorporating all programs within the Heart Institute. This powerful tool has been and will continue to enable research studies aimed at improving the lives of children and adults with congenital heart disease. DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT Risk Stratification in Wolff-Parkinson-White (WPW) Syndrome: The Correlation between Invasive and Noninvasive Testing. This project aims to review a large body of patients with WPW syndrome, correlating results of invasive electrophysiology testing with noninvasive (Holter, exercise, electrocardiography) testing to identify whether any noninvasive markers adequately predict high-risk accessory pathway conduction. Natural History of WPW in a Pediatric Cohort. This study reviews the outcomes of WPW in a large pediatric cohort, spanning more than 30 years of data. Gaurav Arora’s team has reviewed the natural history of these patients with regard to symptoms, documented arrhythmias, and cardiac events (syncope, resuscitated sudden death, sudden death). This study will be the first comprehensive and contemporary investigation of WPW in North America. Major Lectureships and Seminars • Course director, Master Class in Congenital Cardiac Morphology, Children’s Hospital, Robert H. Anderson, October 2011 • “Super Sized Kids: Cholesterol Vivek Allada, MD and Obesity in Interim Division Chief, Pediatric Cardiology Children,” invited speaker, pediatric grand rounds, St. Vincent Medical Center, Erie, Pa., October 2011 225 Professional Affiliations/Society Memberships • American Academy of Pediatrics • American College of Cardiology • American Society of Echocardiography • Section on Cardiology and Cardiothoracic Surgery, American Academy of Pediatrics • Adult Congenital Heart Association WPW Syndrome: Adenosine Response and Correlation with Invasive Electrophysiology Study. This project aims to review the response of pediatric patients with WPW to adenosine and to attempt to correlate with invasive electrophysiology testing results, specifically the risk of rapid antegrade conduction. This project has been submitted for publication. Natural History of Ventricular Ectopy in Pediatric Patients. This study reviews the outcomes of previously healthy children who presented with ventricular ectopy, both isolated premature ventricular complexes (PVCs) and ventricular tachycardia. We reviewed the Children’s Hospital database to create the largest natural history study in a pediatric cohort. Impact of Three-dimensional (3D) Mapping System on Fluoroscopy Time of Atrioventricular Node Reentry Tachycardia (AVNRT) in Pediatric Patients. This study evaluates the impact of a 3D mapping system on fluoroscopy times for ablation of AVNRT in a pediatric cohort. Radiation exposure during ablation has long been the gold standard, but novel imaging systems, like 3D catheter mapping systems, have DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT division of Pediatric Cardiology allowed for reduction of fluoroscopy time in centers using the system for the expressed goal of decreasing fluoroscopy. At Children’s Hospital, the system has been used for five years. In this study, the impact of 3D mapping on fluoroscopy time and efficacy is explored. Double-blind, Randomized, Historical Control Study of the Safety and Efficacy of Eslicarbazepine Acetate Monotherapy in Subjects with Partial Epilepsy Not Well-controlled by Current Antiepileptic Drugs (Sonovian 045). Multicenter study; contributing author; project ongoing 226 A Multicenter, Open-label Study to Investigate the Safety, Tolerability, and Pharmacokinetics of Lacosamide Oral Solution (Syrup) as Adjunctive Therapy in Children with Partial-onset Seizures (Lacosamide 847). Multicenter study Albuterol and Diastolic Hypotension. Contributing author Dexmedetomidine Versus Adenosine: Electrophysiologic Effects and Therapeutic Use for Terminating Supraventricular Tachycardia. Coinvestigator Major Lectureships and Seminars • “Cost-effective Analysis of ICDs in Pediatric Dilated Cardiomyopathy,” invited talk, Heart Rhythm Society Meeting, May 2012 division of Pediatric Cardiology Professional Affiliations/Society Memberships Advisory Committee Memberships Professional Affiliations/Society Memberships • Heart Rhythm Society • Pediatric and Congenital Electrophysiology Society • Pittsburgh Electrophysiology Society • Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, American Heart Association • Writing groups, Guidelines for the Diagnosis of Rheumatic Fever Using the Jones Criteria, Update in the Era of Echocardiography, and Update on Management of Bacterial Endocarditis in Pediatric Population, American Heart Association • President and executive committee, Children’s Hospital Alumni Association • President-elect, Children’s Hospital Medical Staff, 2011–12 • American Academy of Pediatrics • American College of Cardiology • American Heart Association • Society of Critical Care Medicine • Pediatric Cardiac Intensive Care Society • World Society of Pediatric and Congenital Heart Surgery Lee B. Beerman, MD Research Risk Stratification in WPW Syndrome: The Correlation between Invasive and Noninvasive Testing. This project aims to review a large body of patients with WPW syndrome, correlating results of the invasive electrophysiology testing with noninvasive (Holter, exercise, electrocardiography) testing to identify whether any noninvasive markers adequately predict high-risk accessory pathway conduction. Natural History of WPW in a Pediatric Cohort. This study reviews the outcomes of WPW in a large pediatric cohort, spanning more than 30 years of data. We have reviewed the natural history of these patients with regards to symptoms, documented arrhythmias, and cardiac events (syncope, resuscitated sudden death, sudden death). This study will be the first comprehensive and contemporary investigation of WPW in North America. Professional Affiliations/Society Memberships • American Heart Association • Heart Rhythm Society • Pediatric and Congenital Electrophysiology Society • Pittsburgh Electrophysiology Society • Adult Congenital Heart Disease Association Honors • 2012 Peter J. Safar Pulse of Pittsburgh Award, a lifetime achievement award, sponsored by the American Heart Association, 2011 Constantinos Chrysostomou, MD Research Constantinos Chrysostomou has led the introduction of dexmedetomidine in the pediatric population and particularly in children with cardiac disease. Ongoing studies led by Chrysostomou include the following: • A Phase II, Open-label, Multicenter, Escalating Dose Study to Determine Pharmacokinetic and Pharmacodynamic Profile of Dexmedetomidine in Neonates Ages ≥ 28 Weeks to ≤ 44 Weeks Post Gestational Age • Phase III Multicenter Clinical Trial: Dexmedetomidine Safety and Efficacy in the Pediatric Population • Evaluation of Electrocardiographic Effects of Dexmedetomidine in Children with Congenital Heart Disease • Assessment of Pulmonary Artery Pressure and Left Ventricular Function after Administration of Dexmedetomidine • Effect of Dexmedetomidine on Inflammatory Response after Cardiopulmonary Bypass in Children DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT Stephen C. Cook, MD, FACC Research Development of a New Adult Congenital Heart Disease Program: Health Care Appraisal and Needs upon Embarkment. This is a quality-improvement study to obtain an improved understanding of the needs of the adult congenital community in a newly established program and identify problematic areas that may require attention in a tertiary care center. 227 Assessment of the Coronaries and Cerebral Circulation in Adults with Repaired Coarctation. The goal of this study is to assess the prevalence of atherosclerosis and cerebral aneurysms noninvasively in young adults who have undergone prior surgical repair of coarctation of the aorta. Shock, Sexual Function, and Implantable Cardiac Defibrillators (ICDs): An Evaluation of the Adult Congenital Community. This study evaluates sexual function in adults with congenital heart disease without ICDs compared to those with ICDs. Advisory Committee Memberships • Cochair, Research Committee, Adult Congenital Heart Association • Pediatric and Adolescent CTA Committee, Society of Cardiovascular Computed Tomography • Alliance for Adult Research in Congenital Cardiology Major Lectureships and Seminars • “Adults with Congenital Heart Disease,” Department of Pediatrics grand rounds, Children’s Hospital, October 2011 Professional Affiliations/Society Memberships • International Society for Adult Congenital Cardiac Disease • Society for Cardiovascular Magnetic Resonance • Society of Cardiovascular Computed Tomography • American Medical Association • American Academy of Pediatrics • American College of Cardiology DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT division of Pediatric Cardiology Mark DeBrunner, MD Johanna L. Drickman, MD Research Professional Affiliations/Society Memberships Noninvasive assessment of ventricular function, including following heart transplantation • American Academy of Pediatrics • American College of Cardiology • American Physicians Fellowship Major Lectureships and Seminars • “Master Class in Congenital Cardiac Morphology,” course director, Children’s Hospital, Robert H. Anderson, October 2011 Professional Affiliations/Society Memberships • American College of Cardiology Stacey Drant, MD 228 division of Pediatric Cardiology Research Aortic Stiffness in Aortic Root Dilatation. A subgroup of patients with bicuspid aortic valve develop aortic dilatation during childhood and are at increased risk for aortic dissection during adulthood. Patients with other forms of aortic dilatation have been found to have abnormal aortic elastic qualities. Using echocardiography to measure aortic distensibility and stiffness, Stacey Drant’s team is evaluating normal pediatric patients and those with bicuspid aortic valve in an attempt to identify patients who will be at increased risk for future aortic dissection. National Clinical Trial of Beta Blocker Therapy (Atenolol) Versus Angiotension lI Receptor Blocker Therapy (Losartan) in Individuals with Marfan Syndrome. The primary aim of the Marfan trial is to compare the effect of atenolol therapy to that of losartan therapy on the rate of aortic growth and progression of aortic regurgitation. Use of the Myocardial Performance Index Derived by Echocardiography to Identify Rejection in Patients after Heart Transplant. Standard, noninvasive imaging has not proven to be very effective in identifying patients with rejection following heart transplantation. The Myocardial Performance Index, derived through Doppler and tissue Doppler measurements, has been used to measure subtle forms of myocardial dysfunction. This retrospective study utilizes a large transplant database and confirmed that the Myocardial Performance Index calculated with spectral Doppler predicts cardiac transplant rejection. Ongoing studies will utilize tissue Doppler indices to measure Myocardial Performance Index in a prospective fashion. Professional Affiliations/Society Memberships • American Society of Echocardiography DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT Brian D. Feingold, MD, MS Research Listing Strategies for Allosensitized Pediatric Heart Transplant Candidates. Brian Feingold was awarded an NIH KL2 grant in July 2010 to determine historical outcomes for children listed for heart transplantation with a requirement for prospective crossmatch. A comparison of two competing listing strategies for highly allosensitized patients also will be performed. Analyses will be performed with data from the United Network for Organ Sharing and Pediatric Heart Transplant Study databases. Importance of Alloantibodies in Determining Pre- and Post-transplant Outcomes in Pediatric Heart Transplantation. This project has investigated the role of preformed and de novo alloantibodies in determining outcomes before and after pediatric heart transplantation. The prevalence of preformed and de novo antibodies by various detection methods has been established, and the impact of alloantibodies on graft and patient outcomes is currently being defined. The impact of transplanting against a positive donor-specific crossmatch is being evaluated in a prospective cohort of candidates who are known to be presensitized against donor human leukocyte antigens (HLAs). The pilot data have been used to develop the protocol for the Clinical Trials in Organ Transplantation in Children (CTOT-C), for which Feingold is the Children’s Hospital site principal investigator. Use of Cell-bound Complement Activation Product Levels in Detecting Antibody-mediated Rejection in Pediatric Heart Transplantation. This project, supported by a Hillman Innovation Development Transplantation Award, is designed to determine whether cell-bound complement activation products measured on whole blood of pediatric heart transplant recipients can be used as a simple, noninvasive marker of antibody-mediated rejection. Feingold’s research team hypothesizes that cell-bound complement activation products and complement regulatory proteins are useful biomarkers for antibody-mediated rejection after pediatric cardiac transplantation. It is anticipated that this research will allow for accurate diagnosis of antibodymediated rejection, with the potential to predict impending acute antibody-mediated rejection prior to the onset of graft dysfunction. This would enable tailoring of immunosuppression on a case-by-case basis. their grafts in the presence of donor-specific antibody and a positive donor-specific cross-match. Determination of Expression of Blood Group Antigens (ABO) on Cryopreserved Aortic and Pulmonary Artery Homografts. Homografts used in repair of congenital heart defects invariably fail, and the importance of ABO mismatching in this setting is unclear. The research team has therefore studied blood group antigen expression on cryopreserved homografts and have shown that A and B blood group antigens are strongly expressed in the medial and adventitial vaso vasorum. This may have important implications for homograft failure, as well as for the induction of tolerance to blood group antigens when infants are exposed to incompatible blood group antigens in early life. In a continuation of this line of research, there has been no evidence of tolerance to incompatible A and B blood group antigens after homograft placement in infancy. This was found despite persistence of blood group and HLA antigens on explanted homograft specimens, as late as 10 years following implantation. These results were recently published in Human Immunology. Advisory Committee Memberships Epstein-Barr Virus (EBV) Infection in the Immuno-compromised Host. Primary EBV infection is a major cause of morbidity and mortality after pediatric thoracic organ transplantation and frequently is associated with the development of posttransplant lymphoproliferative disorder (PTLD). Feingold serves as a coinvestigator on Diana Metes’ R01 looking at Chronic High EBV Load and Risk of PTLD in Pediatric Thoracic Transplant Patients. • Review Board, United Network for Organ Sharing Region 2 • Fellow Research Day Task Force, American Heart Association Major Lectureships and Seminars • “Pediatric Pulmonary Hypertension,” University of Pittsburgh Department of Pediatrics grand rounds, July 2011 • “Current Management of Pediatric Heart Failure,” Fundación Cardiovascular de Colombia: 3rd Symposium on Pediatric Cardiology and Cardiovascular Surgery, August 2011 • “Key Issues and Experiences in Pediatric Cardiac Transplantation,” Fundación Cardiovascular de Colombia: 3rd Symposium on Pediatric Cardiology and Cardiovascular Surgery, August 2011 • “Innovations in the Monitoring and Treatment of Children Who Require Transplantation,” Fundación Cardiovascular de Colombia: 3rd Symposium on Pediatric Cardiology and Cardiovascular Surgery, August 2011 229 Role of Alloantibodies in Cardiac Transplantation: Intervention, Outcomes, and Mechanisms. Feingold is the Children’s Hospital site primary investigator on this NIH National Institute of Allergy and Infectious Diseases award. This program (CTOT-C) of seven pediatric heart transplant centers seeks to enhance understanding of the role of alloantibodies in pediatric heart transplantation. Antibodies directed against allograft antigens are being increasingly recognized as critical determinants of allograft outcomes in adults. Pediatric heart transplant candidates are frequently allosensitized based on prior exposure to blood products and homografts, and this has led to increased pre- and post-transplant mortality. This research program brings together a group of seven leading heart transplant centers and leading transplantation scientists to study the impact of preformed and de novo alloantibodies on pediatric heart transplant outcomes. Mechanistic studies are being performed to seek explanation for why some children develop graft injury while others appear to “accommodate” DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT division of Pediatric Cardiology Professional Affiliations/Society Memberships • American Heart Association • International Pediatric Transplant Association • American Society of Transplantation • International Society for Heart and Lung Transplantation Honors • KL2 Award from the NIH/National Center for Research Resources Jacqueline Kreutzer, MD, FACC, FSCAI Research 230 Jacqueline Kreutzer’s research interests are in the area of novel technologies for transcatheter intervention including percutaneous valve therapy, device and stent development, and their clinical applications. Kreutzer has been principal investigator for the following projects. The Medtronic Melody Transcatheter Pulmonary Valve Postapproval Study. Kreutzer serves as the principal investigator for this multicenter study on post-market surveillance of the Melody Pulmonary Valve. AGA Atrial Septal Occluder Post-market Study II. A postmarketing surveillance study of an atrial septic defect (ASD) occlusion device placed by cardiac catheterization that is approved by the U.S. Food and Drug Administration (FDA). Congenital Cardiovascular Interventional Study Consortium. A multicenter study designed to compare outcomes between surgery, balloon angioplasty, and intravascular stent placement for recurrent or native coarctation of the aorta. Coarctation of the Aorta Stent Trial (COAST I and COAST II) Multicenter Studies. Multicenter study on the use of CP platinum bare metal stents as well as covered stents for coarctation of the aorta. Congenital Cardiac Catheterization Outcomes Project (C3PO). A multi-institutional quality-improvement project to monitor outcomes of pediatric cardiac catheterization. Advisory Committee Memberships • Congenital Heart Disease Committee, Society of Cardiac Angiography and Intervention • Credentialing Committee, Sub-board of Pediatric Cardiology, American Board of Pediatrics • Scientific Committee, Fourth World Congress of Pediatric Cardiology and Cardiac Surgery DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT division of Pediatric Cardiology • Congenital Cardiovascular Interventional Study Consortium • Writing group for the American Heart Association Scientific Statement, “Anticoagulation in Children” • Council on Cardiovascular Disease in the Young, American Heart Association • Council on Cardiovascular Radiology and Intervention, American Heart Association Major Lectureships and Seminars • Invited speaker, neonatology grand rounds, MageeWomens Hospital, Pittsburgh, Pa., July 2011 • Invited faculty, Pediatric and Adult International Cardiac Symposium (PICS-AICS) 2011, Boston, Mass., July 2011 • Invited speaker, pediatric grand rounds, Charleston Area Medical Center Women and Children’s Hospital, Charleston, W.Va., August 2011 • “Update in Pediatric Interventional Cardiology,” invited speaker, McGowan Institute Research Retreat lecture, March 2012 • Invited speaker, American College of Cardiology 59th Scientific Sessions, Chicago, Ill., March 2012 • Invited speaker/live case session moderator, PICS-AICS, Chicago, Ill., April 2012 • “Tips and Tricks in Congenital and Structural Interventions,” invited faculty, September 2012 Professional Affiliations/Society Memberships • American College of Cardiology • Congenital Heart Disease and Pediatric Cardiology Section, American College of Cardiology • Women in Cardiology Section, American College of Cardiology • American Heart Association • Society for Cardiac Angiography and Interventions • Sociedad Argentina de Cardiología • Sociedad Latina de Cardiología HONORS • The Heart Center Excellence in Teaching Award, 2011 Lizabeth Lanford, MD Professional Affiliations/Society Memberships • American Society of Echocardiography • Prevention of RSV infection in high-risk patients with congenital heart disease • Outcomes after pediatric heart transplantation Elisa Marcuccio, MD Professional Affiliations/Society Memberships Research Noninvasive Measurement of Cardiac Output during Exercise in Children with Tetralogy of Fallot. Elisa Marcuccio is comparing cardiac output data in patients with repaired Tetralogy of Fallot using the technique in the study above with normal subjects. Francis M. McCaffrey, MD Linda Russo, MD Professional Affiliations/Society Memberships • American Society of Echocardiography Research Clinical Trial of Coenzyme Q10 and Lisinopril in Muscular Dystrophies. This is a multicenter study that enrolls patients with muscular dystrophies early in their disease, before the onset of cardiomyopathy, into a coenzyme Q10/lisinopril arm versus a traditional treatment control. Echocardiographic monitoring is used to determine whether they qualify for enrollment and to track cardiac function. Cardiac Outcome Measures in Children with Muscular Dystrophy. This is an extension of the above study and compares inter-observer variability for many of the measures that are being used for the coenzyme Q10/lisinopril study to help determine which of those measures would be the most reproducible across multiple institutions. Noninvasive Exercise Cardiac Output in Children and Young Adults. The team is collecting normative data on a new exercise measurement and eventually will compile, summarize, and report the data. Noninvasive Measurement of Cardiac Output During Exercise in Children with Tetralogy of Fallot. The team is comparing cardiac output data in patients with repaired Tetralogy of Fallot using the technique in the study above with normal subjects. Research Professional Affiliations/Society Memberships Lizabeth Lanford’s research interest lies in cardiovascular abnormalities and pulmonary hypertension in children with sickle cell disease. • American Society of Echocardiography Major Lectureships and Seminars Research • Course director, “Master Class in Congenital Cardiac Morphology,” Children’s Hospital, Robert H. Anderson, “Echocardiographic Features of Congenital Heart Disease,” October 2011 • American Academy of Pediatrics • American Heart Association • International Pediatric Transplant Association • International Society for Heart and Lung Transplantation Susan A. Miller, MD Susan Miller’s research interests include the following: • AGA Atrial Septal Occluder Post-market Study II, which is a postmarketing surveillance study of an FDA-approved ASD occlusion device placed by cardiac catheterization 231 Fred S. Sherman, MD, MBA Research Fred Sherman’s research interests include computer-assisted navigation applied to fetal cardiac intervention and the examination of causative factors in peripartum cardiomyopathy. He is developing clinical pathways for fetal supraventricular tachycardia and participated in developing a clinical pathway for management of the patent ductus arteriosus. A paper, “Management of the Single Ventricle Patient during Labor Delivery” is in press in the Journal of Clinical Anesthesiology. Professional Affiliations/Society Memberships • American Academy of Pediatrics • American Society of Echocardiography • American Heart Association • American College of Cardiology • Society of Pediatric Echocardiography Sara Trucco, MD Research Sara Trucco’s research involves being a: • Subinvestigator for study titled, Comparison between Surgical Versus Balloon Angioplasty Versus Intravascular Stent Placement for Recurrent or Native Coarctation of the Aorta • Subinvestigator for a study titled, Covered Cheatham Platinum Stent for Prevention or Treatment of Aorta Wall Injury Associated with Aortic Coarctation (COAST II) • Subinvestigator for a study titled, Amplatzer Atrial Septal Defect Occluder Post-marketing Study II-Clinical Study through AGA Medical Corporation Professional Affiliations/Society Memberships • American Heart Association DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT division of Pediatric Cardiology division of Pediatric Cardiology T TEACHING ACTIVITIES he pediatric cardiology faculty plays an active role in the teaching of medical students, residents, fellows, nurse practitioners, ultrasound students, and adult cardiology trainees. There is an ACGME-accredited fellowship training program that has trained more than 60 pediatric cardiologists and currently is training eight fellows. In addition, predoctoral and postdoctoral trainees form part of the cardiovascular research programs of the Cardiology Division and the Heart Center. In addition, the pediatric cardiology faculty hosted the fifth annual Cardiology Morphology Course, in collaboration with world-renowned cardiac pathologist Robert Anderson. THREE-YEAR BIBLIOGRAPHY 2010 232 Chrysostomou C, Komarlu R, Lichtenstein S, Shiderly D, Arora G, Orr R, Wearden PD, Morell VO, Munoz R, Jooste EH. Electrocardiographic effects of dexmedetomidine in patients with congenital heart disease. Intensive Care Med, 36(5):836-42, 2010. Crestanello JA, Cook S, Daniels C, Hitchcock CL, Sai-Sudhakar C. Medial necrosis in aortic root aneurysm after repair of tetralogy of Fallot. J Card Surg, 25(2):230-2, 2010. Davies ML, Xu S, Lyons-Weiler J, Rosendorff A, Webber SA, Wasil LR, Metes D, Rowe DT. Cellular factors associated with latency and spontaneous Epstein-Barr virus reactivation in B-lymphoblastoid cell lines. Virology, 400:53-67, 2010. Doshi AA, Cook SC, Hummel JD. Implantation of a bi-ventricular pacing system in the setting of dextrocardia with situs inversus totalis. Indian Pacing Electrophysiol J,10(1):58-61, 2010. Feingold B, Arora G, Webber SA, Smith KJ. Cost-effectiveness of implantable cardioverter-defibrillators in children with dilated cardiomyopathy. J Cardiac Fail, 16(9):734-41, 2010. Foerster SR, Canter CE, Cinar A, Sleeper LA, Webber SA, Pahl E, Kantor PF, Alvarez JA, Colan SD, Jeffries JL, Lamour JM, Margossian R, Messere JR, Rusconi PG, Shaddy RE, Towbin JA, Wilkinson JD, Lipshultz SE. Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: An outcomes study from the pediatric cardiomyopathy registry. Circ Heart Fail, 3:689-97, 2010. DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT Holzer R, Marshall A, Kreutzer J, Hirsch R, Chisolm J, Hill S, Galantowicz M, Phillips A, Cheatham J, Bergerson L. Hybrid procedures: Adverse events and procedural characteristics— Results of a multi-institutional registry. Congenit Heart Dis, 5(3):233-42, 2010. Johnson MR, Haft J, Kinder D, Webber SA, Meyer K, Shervin C, Pitts N, Dyke DB. Heart transplantation in the United States 1999-2008. Am J Transplant, 10(4 Pt 2):1035-46, 2010. Jooste EH, Muhly WT, Ibinson JW, Suresh T, Damian D, Phadke A, Callahan P, Miller S, Feingold B, Lichtenstein SE, Cain JG, Chrysostomou C, Davis PJ. Acute hemodynamic changes following rapid intravenous bolus dosing of dexmedetomidine in pediatric heart transplant patients undergoing routine cardiac catheterization. Anesth Analg,111(6):1490-6, 2010. Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J, Valente AM, Earing MG, Lui G, Gersony DR, Cook S, Ting JG, Nickolaus MJ, Webb G, Landzberg MJ, Broberg CS; for the Alliance for Adult Research in Congenital Cardiology (AARCC). Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: A multi-institutional study. Circulation, 868-75, 2010. Lazol JP, Lichtenstein SE, Jooste EH, Shiderly D, Kudchadker NA, Tatum GH, Orr RA, Wearden PD, Morell VO, Munoz RA, Chrysostomou C. Effect of dexmedetomidine on pulmonary artery pressure after congenital cardiac surgery: A pilot study. Pediatr Crit Care Med, 11(5):589-92, 2010. Manrique AM, Arroyo M, Lin Y, El Khoudary SR, Colvin E, Lichtenstein S, Chrysostomou C, Orr R, Jooste E, Davis P, Wearden P, Morell V, Munoz R. Magnesium supplementation during cardiopulmonary bypass to prevent junctional ectopic tachycardia after pediatric cardiac surgery: A randomized controlled study. J Thorac Cardiovasc Surg, 139(1):162-9, 2010. Moguillansky D, Rome JJ, Schneider H, Kreutzer J. Role of high-pressure balloon valvotomy for resistant pulmonary valve stenosis. Congenital Heart Disease, 5(2):134-40, 2010. Newman B, Feinstein JA, Cohen RA, Feingold B, Kreutzer J, Patel H, Chan F. Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia. Pediatr Radiol, 40(7):1222-30, 2010. Ohmann EL, Burckart GJ, Brooks MM, Chen Y, Pravica V, Girnita DM, Zeevi A, Webber SA. Genetic polymorphisms influence mycophenolate mofetil related adverse events in pediatric heart transplant patients. J Heart Lung Transpl, 29:509-16, 2010. Ohmann EL, Burckart GJ, Chen Y, Pravica V, Brooks MM, Zeevi A, Webber SA. Inosine 5’-monophosphate dehydrogenase 1 haplotypes and association with mycophenolate mofetil gastrointestinal intolerance in pediatric heart transplant patients. Pediatric Transplantation,14:891-5, 2010. Ohmann EL, Brooks MM, Webber SA, Girnita DM, Ferrell RE, Burckart GJ, Chinnock R, Canter C, Addonizio L, Bernstein D, Kirklin JK, Naftel D, Zeevi A. Association of genetic polymorphisms and risk of late posttransplant infection in pediatric heart recipients. J Heart Lung Transplant, 29:1342-51, 2010. Parent B, Munoz R, Shiderly D, Chrysostomou C. Use of dexmedetomidine in sustained ventricular tachycardia. Anaesth Intensive Care, 38(4):781, 2010. Sanchez de Toledo J, Gunawardena S, Munoz R, Orr R, Berry D, Sonderman S, Krallman S, Shiderly D, Wang L, Wearden P, Morell VO, Chrysostomou C. Do neonates, infants and young children need a higher dose of enoxaparin in the cardiac intensive care unit? Cardiol Young, 20(2): 138-43, 2010. Sanchez de Toledo J, Chrysostomou C, Wearden PD. Acute compartment syndrome in a patient on extracorporeal support: Utility of near-infrared spectroscopy. J Cardiothorac Vasc Anesth, September 8, 2010. [Epub ahead of print] Sanchez de Toledo J, Munoz R, Landsittel D, Shiderly D, Yoshida M, Komarlu R, Wearden P, Morell VO, Chrysostomou C. Diagnosis of abnormal diaphragm motion after cardiothoracic surgery: Ultrasound performed by a cardiac intensivist vs. fluoroscopy. Congenit Heart Dis, 5(6):565-72, 2010. Seale AN, Uemura H, Webber SA, Partridge J, Roughton M, Ho SY, McCarthy KP, Jones S, Shaughnessy L, Sunnegardh J, Hanseus K, Berggren H, Johansson S, Rigby ML, Keeton BR, Daubeney PEF. Total anomalous pulmonary venous connection: Morphology and outcome from an international population-based study. Circulation, 122:2718-26, 2010. Valente AM, Bhatt A, Cook S, Earing MG, Gersony DR, Aboulhosn J, Opotowsky AR, Lui G, Gurvitz M, Graham D, Fernandes SM, Khairy P, Webb G, Gerhard-Herman M, Landzberg M. The CALF (Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients) Study. JACC, 56:144-50, 2010. Verheart D, Arruda J, Thavendiranathan P, Cook SC, Raman SV. Truncus arteriosus with aortic arch interruption: Cardiovascular magnetic resonance findings in the unrepaired adult. J Cardiovas Magn Reson, 12(1):16, 2010. 2011 Alvarez JA, Orav EJ, Wilkinson JD, Fleming LE, Lee DJ, Sleeper LA, Rusconi PG, Colan SD, Hsu DT, Canter CE, Webber SA, Cox GF, Jeffries JL, Towbin JA, Lipshulz SE. Competing risks for death and cardiac transplantation in children with dilated cardiomyopathy: Results from the Pediatric Cardiomyopathy Registry. Circulation, 124:814-23, 2011. Bergersen L, Gauvreau K, Marshall A, Kreutzer J, Beekman R, Hirsch R, Foerster S, Balzer D, Vincent J, Hellenbrand W, Holzer R, Cheatham J, Moore J, Lock J, Jenkins K. Proceduretype risk categories for pediatric and congenital cardiac catheterization. Circ Cardiovasc Interv, 4(2):188-94, 2011. Białkowski J, Glowacki J, Zabal C, Garcia-Montes A, Bermudez-Canete R, Flores-Arizmendi R, Sagado-Sandova A, Diaz-de Leon H, Menacho-Delgadillo R, Kreutzer J. Patent ductus arteriosus at low and high altitudes: Anatomical and hemodynamic features and their implications for transcatheter closure. Kardiol Pol, 69(5):431-6, 2011. Chrysostomou C, De Toledo JS, Wearden P, Jooste EH, Lichtenstein SE, Callahan PM, Suresh T, O’Malley E, Shiderly D, Haney J, Yoshida M, Orr R, Munoz R, Morell VO. Perioperative use of dexmedetomidine is associated with decreased incidence of ventricular and supraventricular tachyarrhythmias after congenital cardiac surgery. Ann Thorac Surg, 92(3):964-72, 2011. Feingold B, Raval J, Galambos C, Yazer M, Zeevi A, Bentlejewski C, Morell VO, Wearden PD, Webber SA. Tolerance to incompatible ABO blood group antigens is not observed following homograft implantation. Human Immunology, June 15, 2011. [Epub ahead of print] Feingold B, Irving C, Tatum GH, Webber SA. Prognostic significance of recurrent grade 1B rejection in the first year after pediatric cardiac transplantation: A case for reinstatement of the 1B rejection grade. Pediatr Transplant,15:589-93, 2011. Gaur L, Madan S, Morell V, Arora G. Formation of ventricular aneurysm after endocarditis in a child with methicillinresistant Staphylococcus aureus (MRSA) bacteremia. Pediatr Cardiol, July 26, 2011. [Epub ahead of print] Girnita DM, Ohmann EL, Brooks MM, Webber SA, Burckart GJ, Ferrell RE, Ranganathan S, Chinnock R, Canter C, Addonizio L, Bernstein D, Kirklin JK, Naftel DC, Zeevi A. Gene polymorphisms impact the risk of rejection with hemodynamic compromise: A multicenter study. Transplantation, 91:1326-32, 2011. Guglani L, Tadros S, Morell VO, Spahr JE, Webber SA, Kurland G. Lobar displacement following pediatric heart-lung transplantation. Pediatric Transplantation, January 12, 2011. [Epub ahead of print] Holzer RJ, Gauvreau K, Kreutzer J, Leahy R, Murphy J, Lock JE, Cheatham JP, Bergersen L. Balloon angioplasty and stenting of branch pulmonary arteries: Adverse events and procedural characteristics: Results of a multiinstitutional registry. Circ Cardiovasc Interv, 4(3):287-96, 2011. 233 Komarlu R, Beerman LB, Freeman D, Arora G. Fetal and neonatal presentation of long QT syndrome. PACE, March 14, 2011. [Epub ahead of print] Lapage MJ, Reed JH, Collins KK, Law IH, Pilcher TA, Tanel RE, Anderson CC, Young ML, Emmel M, Paul T, Blaufox AD, Arora G, Saul JP. Safety and results of cryoablation in patients <5 years old and/or <15 kilograms. Am J Cardiol, 108(4):565-71, 2011. Lawrence KS, Stilley CS, Pollock JA, Webber SA, Quivers E. A familycentered educational program to promote independence in pediatric heart transplant recipients. Progress in Transplantation, 21:61-6, 2011. Macedo C, Webber SA, Donnenberg A, Popescu I, Hua Y, Green M, Rowe D, Smith L, Brooks MM, Metes D. EBV-specific CD8+ T Cells from asymptomatic pediatric thoracic transplant patients carrying chronic high ebv loads display contrasting features: Activated phenotype and exhausted function. J Immunology, 186:5854-62, 2011. Misra N, Webber SA, DeGroff CG. Adult definitions for dyssynchrony are inappropriate for pediatric patients. Echocardiography, 28:468-74, 2011. DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT division of Pediatric Cardiology Parent B, Wearden P, Kounis GA, Chrysostomou C. Kounis syndrome or allergic coronary vasospasm in a two-year old. Congenital Heart Disease, March 21, 2011. [Epub ahead of print] Picarsic J, Jaffe R, Mazariegos G, Webber SA, Ellis D, Green MD, ReyesMúgica M. Post-transplant Burkitt lymphoma is a more aggressive and distinct form of PTLD. Cancer, March 28, 2011. [Epub ahead of print] Sanchez MA, DeBrunner M, Cox E, Caldwell R. Acquired toxoplasmosis after orthotopic heart transplantation in a sulfonamide-allergic patient. Pediatric Cardiology, 32(1):91-3, 2011. 234 Trucco SM, Jaeggi E, Cuneo B, MoonGrady AJ, Silverman E, Silverman N, Hornberger LK. Use of intravenous gamma globulin and corticosteroids in the treatment of maternal autoantibodymediated cardiomyopathy. J Am Coll Cardiol, 57(6):715-23, 2011. DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT Trucco SM, Barnoya J, Larrazabal LA, Castañeda A, Teitel DF. Detection rates of congenital heart disease in Guatemala. Cardiol Young, 21(2): 153-60, 2011. Zaoutis TE, Webber SA, Naftel DC, Chrisant MA, Kaufman B, Bennett Pearce F, Spicer R, Dipchand AI. Invasive fungal infections in pediatric heart transplant recipients: Incidence, risk factors and outcomes. Pediatr Transplant,15:465-9, 2011. 2012 Beerman L, Kreutzer J, Allada V. Chapter 5: Cardiology. In: Atlas of Pediatric Physical Diagnosis, sixth edition. Zitelli BJ, McIntyre S, Nowalk A (Eds.). Baltimore, MD: Mosby-Wolfe, 2012. West L, Beerman LB, Arora G. “The natural history of ventricular ectopy in children.” Presented at American Heart Association Fellows’ Research Day. Pittsburgh, Pa., February 17, 2012. division of Pediatric Cardiology Wackel P, Beerman LB, Arora G. “Wolff-Parkinson-White and adenosine response in pediatric patients.” Presented at American Heart Association Fellows’ Research Day. Pittsburgh, Pa., February 17, 2012. Beach C, Beerman LB, Sukits V, Mazzocco S, Hangard A, Brooks MM, Arora G. “Impact of three-dimensional mapping system on fluoroscopy times in pediatric AVNRT: Mid-term results. Presented at American Heart Association Fellows’ Research Day.” in Pittsburgh, Pa., February 17, 2012. Zeevi A, Marrari M, Feingold B, Webber SA, Duquesnoy RJ. HLA epitope analysis to assess complement and noncomplement binding donor specific antibody repertoire in a pediatric heart transplant recipient. Human Immunol, 73:48-51, 2012. Holzer RJ, Gauvreau K, Kreutzer J, Trucco SM, Torres A, Shahanavaz S, Bergersen L. Safety and efficacy of balloon valvuloplasty for isolated pulmonary valve stenosis: A multicenter experience. Catheter Cardiovasc Interv, 2012. Ying X, Reyes-Mugica M, Galambos C, Zeevi A, Miller SA, Webber SA, Feingold B. Utility of routine C4d staining in the first year after pediatric heart transplantation. Presented at the International Society for Heart and Lung Transplantation, Prague, Czech Republic, April 2012. Seale AN, Carvalho JS, Gardiner HM, Mellander M, Roughton M, Simpson J, Tometzki A, Uzun O, Webber SA, Daubeney PEF. Total anomalous pulmonary venous connection: Impact of prenatal diagnosis. Ultrasound Obstet Gynecol, Jan 20, 2012. [Epub ahead of print] Marcuccio E, Arora G, McCaffrey F. Noninvasive measurement of cardiac output during exercise in children with tetralogy of Fallot. Pediatr Cardiol, 33(7):1165-70, 2012. Pahl E, Sleeper LA, Canter CE, Hsu DT, Lu M, Webber SA, Colan SD, Kantor PF, Everitt MD, Towbin JA, Jefferies JL, Kaufman BD, Wilkinson JD, Lipshultz SE; for the Pediatric Cardiomyopathy Registry. Incidence and risk factors for sudden cardiac death in children with dilated cardiomyopathy: A report from the pediatric cardiomyopathy registry. J Am Coll Cardiol, 59:607-15, 2012. Turner Z, Lanford L, Webber SA. Oral budesonide as a therapy for protein-losing enteropathy in patients having undergone fontan palliation. Congenital Heart Dis, 7:24-30, 2012. 235 Wiesmayr S, Webber SA, Macedo C, Popescu I, Smith S, Luce J, Metes D. Decreased NKp46 and NKG2D and elevated PD-1 are associated with altered NK-cell function in pediatric transplant patients with PTLD. Eur J Immunol, 42:541-50, 2012. DEPARTMENT OF PEDIATRICS 2012 ANNUAL REPORT