on March 8, 2004
Published online before print March 8, 2004, doi: 10.1161/01.CIR.0000121738.88273.43
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Submitted on September 18, 2003
From the Department of Cardiology and Angiology (T.W., M.P., C.W., T.A., P.G., O.A., M. Block, M. Borggrefe, G.B., D.B.) and the Department of Thoracic and Cardiovascular Surgery (T.D.T.T., R.S., H.H.S.), University Hospital of Münster; and Institute for Arteriosclerosis Research at the University of Münster (T.W., G.B.), Münster, Germany. Dr Borggrefe is currently at the First Department of Medicine, University Hospital Mannheim, University of Heidelberg, Germany, and Dr Block is currently at the Department of Cardiology, Stiftsklink Augustinum, München, Germany. * To whom correspondence should be addressed. E-mail: wichtet{at}uni-muenster.de.
Background--Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of ventricular tachycardia (VT) and cardiac arrest in young patients. We hypothesized that treatment with implantable cardioverter/defibrillators (ICDs) is safe and improves the long-term prognosis of ARVC patients at high risk of sudden death. Methods and Results--Sixty patients with ARVC (aged 43±16 years) were treated with transvenous ICD systems. Despite a higher number of right ventricular sites tested for adequate lead positions (P<0.05), lower R-wave amplitudes (P<0.001) were achieved in ARVC patients compared with other entities. During follow-up of 80±43 months (396 patient-years), event-free survival was 49%, 30%, 26%, and 26% for appropriate ICD therapies and 79%, 64%, 59%, and 56% for potentially fatal VT (>240 bpm) after 1, 3, 5, and 7 years, respectively. Multivariate analysis identified extensive right ventricular dysfunction as an independent predictor of appropriate ICD discharge. Fifty-three adverse events occurred in 37 patients during the perioperative (n=10) or follow-up (n=43) period, mainly related to the leads (n=31 in 21 patients). No lead perforation was observed. Freedom from adverse events was 90%, 78%, 56%, and 42% and freedom from lead-related complications was 95%, 85%, 74%, and 63% after 1, 3, 5, and 7 years, respectively. Conclusions--These results strongly suggest an improvement in long-term prognosis by ICD therapy in high-risk patients with ARVC. However, meticulous placement and long-term observation of transvenous lead performance with focus on sensing function are required for the prevention and/or early recognition of disease progression and lead-related morbidity during long-term follow-up of ICD therapy in ARVC.
Revised on December 29, 2003
Accepted on January 8, 2004
Implantable Cardioverter/Defibrillator Therapy in Arrhythmogenic Right Ventricular Cardiomyopathy. Single-Center Experience of Long-Term Follow-Up and Complications in 60 Patients
Thomas Wichter MD, FESC*,
Key words: cardiomyopathy • heart arrest • tachyarrhythmias • defibrillators, implantable • arrhythmogenic right ventricular dysplasia
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Circulation 2004 109: 1445-1447.
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