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ESSA HEART AND VASCULAR INSTITUTE ! JULY/AUGUST/SEPTEMBER 2009 CLINICAL LETTER Welcome to the third edition of our clinical newsletter. We certainly appreciate all the wonderful feedback we have received from our readership. This edition features an article by Dr. Ponnathpur on Sudden Cardiac Arres! and the use and indications for Implantable Cardioverter"Defibrillators #ICD’s$. The other article is on Hypertrophic Cardiomyopathy, a potentially lethal problem involving cardiac muscle. Future editions of the newsletter will bring articles such as Presentation and Management of Aortic Dissectio", and The Role of Coronary Stents in the Treatment of Ischemic Heart Diseas#. We also plan to begin to feature articles on non"cardiac thoracic problems. Recently, our cardiac surgical program was honored with a 3"star rating #the maximum grade$ by the Society of Thoracic Surgeons #STS$ for outstanding performance and outcomes. STS employs extremely strict criteria such as mortality, morbidities #stroke, infection$, length of stay, etc. in evaluating cardiovascular programs in the United States. When it is realized that the ESSA Heart and Vascular Institute has been in existence for about only two years, this grand recognition by the STS is worth a celebration. The success of this cardiothoracic program is truly an attestation to the tireless e%orts of a dedicated, tenacious team of administrators and clinical sta%" motivated by excellence in patient care. There is no longer a reason for the people in this community to leave the area in order receive cardiovascular and thoracic care. We will continue to provide world"class care to you. ! ! ! ! ! ! ! ! ! Have a happy summer! ! ! ! ! ! ! ! ! ! Nche Zama MD, PhD THIS MONTH AT ESSA HEART AND VASCULAR INSTITUTE: Our Physician Referral Receptio! July 16 at 6PM Come join us that evening as the Cath Lab hosts a brief lecture by Dr. Zama, an open house, and re$eshments....Questions, please ca% 570&422&8393 POCONO MEDICAL CENTER! PAGE 1 ESSA HEART AND VASCULAR INSTITUTE ! JULY/AUGUST/SEPTEMBER 2009 SUDDEN CARDIAC ARREST "SCA# AND “SHOCKBOXES” "ICDS# DR. VIDYA PONNATHPUR MD, FACC Department of Cardiology at PMC Sudden Cardiac Arrest #SCA$, also known as Sudden Cardiac Death #SCD$ is defined as the unexpected natural death from a cardiac cause within a short time period from the onset of symptoms" an “electrical accident of the heart”. SCA is responsible for 400,000 deaths a year in the U.S. Despite our growing knowledge about the mechanisms and markers of this disease, SCA remains di&cult to treat because the first symptom is often death. Several risk factors have been identified for SCA. Most of them are the usual risk factors for heart disease: smoking, inactivity, obesity, advanced age, hypertension, elevated serum cholesterol and glucose intolerance. About half of SCA patients have myocardial scars or active coronary lesions. A low left ventricular ejection fraction #LVEF$ is a very important risk factor that a%ects people with and without CAD. An LVEF less than or equal to 35' significantly increases the risk of SCA. SCA typically involves a malignant arrhythmia. This requires a substrate or alternate conduction pathway in the heart and a triggering event. It must be noted, however, that SCA can also be the result of a bradyarrhythmia, which has been estimated to occur in about 10' of patients. Hereditary disorders such as Long QT Syndrome #LQTS$ and Brugada Syndrome have been subjects of considerable interest as causes of SCA. Unfortunately, no drug class has been able to convincingly reduce the risk of SCA. On the contrary, many drugs, especially antiarrhythmics, increased deaths in clinical trials. Many trials have established the role of implantable cardioverter"defibrillators #ICDs$ in patients with low LVEF. Three trials" MADIT, MADIT II, and SCD&HeFT have made ICDs the mainstay of device therapy, reducing SCA in patients having a reduced LVEF, less than or equal to 35'. The MADIT trial showed a 50' reduction in mortality in patients with ischemic cardiomyopathy with ICDs, compared to medical therapy. This study required an electrophysiology #EP$ study for inducibility of ventricular arrhythmia. continued on p3 POCONO MEDICAL CENTER! PAGE 2 ESSA HEART AND VASCULAR INSTITUTE ! JULY/AUGUST/SEPTEMBER 2009 SUDDEN CARDIAC ARREST "SCA# AND“SHOCKBOXES” "ICDS#$ continued %om p2 The MADIT II trial did not require an EP study. These patients were simply post"MI patients with LVEF less than or equal to 30'. ICDs significantly reduced the risk of all"cause mortality by 31'. The SCD"HeFT is the largest device trial. Both ischemic and nonischemic cardiomyopathy #LVEF less than or equal to 35'$ patients with NYHA Class II or III symptoms were included. This trial showed that ICDs reduced all"cause mortality by 23' compared to Amiodarone or a placebo. In fact, Amiodarone did as well as the placebo. Michel Mirowski #1924" 1990$ is generally credited as the inventor of the ICD. He was the director of the Coronary Care Unit at Baltimore Sinai Hospital in 1968. He published the first manuscript on ICDs, and the medical community’s response was one of denunciation. In 1980, however, the first human ICD implant occurred. By 1985, 800 devices had been implanted in the U.S. and Europe. The FDA gave the clearance for the first ICD in 1985. Over the past 20 years the devices have become smaller and have better diagnostics. A modern ICD compared to one $om 1987 It is very important to identify patients who are at risk for SCA and evaluate them for the possibility of prophylactic ICDs #for primary prevention$. It is much easier for secondary prevention to implant ICDs as these patients would have survived a near fatal event. In fact, no patient who has survived a cardiac arrest secondary to a malignant arrhythmia should leave the hospital without an evaluation for ICD implantation #if clinically appropriate$ Case Study 1. A 67 year old male with a 4 year history of nonischemic cardiomyopathy, LVEF of 15', had been evaluated for ICD implantation at a di%erent institution but had refused the ICD. Patient was admitted with congestive heart failure exacerbation on optimal medical therapy. During this admission the patient agreed to proceed with prophylactic ICD implantation which was done. He was discharged home and was readmitted 2 weeks later with 4 ICD shocks. ICD interrogation revealed that he had a malignant ventricular arrhythmia and ICD therapy saved his life. continued on p4 POCONO MEDICAL CENTER! PAGE 3 ESSA HEART AND VASCULAR INSTITUTE ! JULY/AUGUST/SEPTEMBER 2009 SUDDEN CARDIAC ARREST "SCA# AND“SHOCKBOXES” "ICDS#$ continued %om p3 #It is important to note that inappropriate ICD therapy can occur in 20' to 25' of patients secondary to supraventricular arrhythmias$ Case Study 2. A 60 year old black female was a survivor of out"of"hospital cardiac arrest secondary to ventricular fibrillation. Evaluation revealed nonischemic cardiomyopathy, LVEF of 25'. She was scheduled for ICD implantation for secondary prevention of SCA. Unfortunately this had to be postponed due to bleeding issues and bilateral upper extremity thrombosis as well as acute renal failure. She made a remarkable recovery over the next 2 to 3 months and was sent home with a LifeVest #external defibrillator$. After rehab and recovery, she successfully underwent ICD implantation and is doing well. INDICATIONS FOR IMPLANTABLE CARDIOVERTER/DEFIBRILLATOR 'ICD( A patient, at risk for sudden cardiac death based on at least one of the criteria marked below, has been receiving Optimal Medical Therapy and has reasonable expectation of survival with good functioning status for more than one year can be considered for this procedure. For Primary Prevention ICD! with history of CABG or PCI the waiting period is 90 days, for post!MI patients, the waiting period is 40 days. ! ! ! ! ! ! 1 2 3. 4. 5. ! ! ! Criteria For Secondary Prevention: Cardiac arrest due to ventricular tachycardia #VT$ or ventricular fibrillation #VF$ Congenital High risk for VT/VF Spontaneous VT/VF, spontaneous or induced by electrophysiology study #EPS$ Hemodynamically unstable VT Syncope after extensive cardiac evaluation and deemed clinically at risk for SCA ! 1. 2. 3. 4. ! ! ! ! Criteria for Primary Prevention: Ejection fraction #EF$ <30'. Prior MI>40 days. NYHA Class I LVEF 31"40'. NYHA Class I"III. NSVT, CAD, prior MI, and inducible sustained VT/VF by EPS LVEF< or = 35'. NYHA Class II"III. History of MI Non ischemic dilated cardiomyopathy #NIDCM$ #medically managed for > 3 months$ LVEF , or = to 35'. POCONO MEDICAL CENTER! PAGE 4 ESSA HEART AND VASCULAR INSTITUTE JULY/AUGUST/SEPTEMBER 2009 ! HYPERTROPHIC CARDIOMYOPATHY DR NCHE ZAMA MD, PHD Department of Cardiothoracic Surgery at PMC Hypertrophic Cardiomyopathy #HCM$ is a disease characterized by hypertrophy, the thickening of the left ventricular myocardium #heart muscle$. This thickening can be obstructive and impede the normal flow of blood from the heart. Characteristically, patients with this disorder have supranormal systolic function as well as impaired diastolic relaxation and compliance. This disease accounts for a significant number of sudden cardiac deaths in all ages and is one of the leading causes of cardiac death in young and active people, especially athletes. Cardiomyopathy in HCM is associated with hypertrophy of sarcomeres #contractile units$ and a loss of the normal cellular arrangement in myocardial muscle, which leads to myocardial disarray. The cause of HCM is unknown. Patients can present with either a familial form which follows an autosomal dominant pattern of inheritance with variable penetrance, or a sporadic form. Hypertrophy is usually asymmetric, involving the interventricular septum. This is in contrast to symmetric concentric hypertrophy that is associated with aortic valve stenosis or hypertension. Oftentimes, dynamic left ventricular outflow obstruction due to systolic anterior motion #SAM$ of the mitral valve anterior leaflet is encountered can can lead to serious symptoms. Occasionally, ventricular wall thickening and systolic compression of coronary arteries may lead to critical coronary insu&ciency involving intramuscular branches. Consequently, the patient can su%er ischemia and infarction #heart attack$ Many patients with HCM are predisposed to atrial and ventricular arrhythmias. Symptomatology Symptoms associated with HCM include: Dyspnea #shortness of breath$ with exertion Fatigue Angina #chest pain or pressure$ Presyncope, syncope #passing out$ Palpitations Diagnosis The diagnosis of HCM can be reached after a complete history and physical examination followed by an echocardiogram. Cardiac catheterization is indicated if the patient is in need of surgery for HCM. ! ! ! ! ! POCONO MEDICAL CENTER! ! ! ! ! ! ! continued on p6 PAGE 5 ESSA HEART AND VASCULAR INSTITUTE ! JULY/AUGUST/SEPTEMBER 2009 HYPERTROPHIC CARDIOMYOPATHY&continued $om p5 Treatment The treatment of HCM depends on symptoms. Medical management does provide symptomatic relief for most patients. Mainstay of medical therapy consists of beta"blockers and calcium channel blockers. Dual chamber cardiac pacing has been used but the results of this modality have not been encouraging. Percutaneous catheter myocardial ablation has been described to relieve left ventricular outflow tract obstruction in patients with HCM. In this procedure, ethanol is injected in the coronary artery to cause a controlled septal myocardial infarction. Because of many important associated complication such as complete heart block requiring a permanent pacemaker, this treatment option is not very popular. ! ! ! ! ! ! ! ! ! ! ! Normal heart Hypertrophic Cardiomyopathy Many surgical techniques have been proposed to relieve left ventricular outflow tract obstruction associated with HCM. The most commonly employed techniques include Mitral Valve Replacement and Transaortic Septal Myectomy. Replacement of the mitral valve with a low profile prosthetic valve relieves the outflow tract obstruction by eliminating systolic anterior motion due to the anterior mitral valve leaflet. Myectomy provides long"lasting symptomatic relief to patients who have failed medical therapy and have documented outflow tract obstruction at rest or on provocation. It can be done safely with minimal complications. POCONO MEDICAL CENTER! PAGE 6 ESSA HEART AND VASCULAR INSTITUTE POCONO MEDICAL CENTER JULY/AUGUST/SEPTEMBER 2009 PAGE 7 ESSA HEART AND VASCULAR INSTITUTE JULY/AUGUST/SEPTEMBER 2009! PLEASE JOIN US FOR: Physician Referral Reception July 16, 6 PM PMC’s Heart and Vascular Institute Cath Lab Grand Rounds “Best Practices for Stroke Management in 2009” July 17, noon PMC’s Stroud/Brodhead Rooms “Clinical Letter”" a publication of ESSA Heart and Vascular Institute at PMC Editor " R. Eileen Butz RN, CCRN Questions/Comments? 570.420.5332