Efficacy of Implantable Cardioverter–Defibrillators for the Prevention of Sudden Death in Patients with Hypertrophic Cardiomyopathy
Barry J. Maron, M.D., Win-Kuang Shen, M.D., Mark S. Link, M.D., Andrew E. Epstein, M.D., Adrian K. Almquist, M.D., James P. Daubert, M.D., Gust H. Bardy, M.D., Stefano Favale, M.D., Robert F. Rea, M.D., Giuseppe Boriani, M.D., N.A. Mark Estes, M.D., Paolo Spirito, M.D., Susan A. Casey, R.N., Marshall S. Stanton, M.D., and Sandro Betocchi, M.D.
Background Hypertrophic cardiomyopathy is a genetic diseaseassociated with a risk of ventricular tachyarrhythmias and suddendeath, especially in young patients.
Methods We conducted a retrospective multicenter study of theefficacy of implantable cardioverter–defibrillators inpreventing sudden death in 128 patients with hypertrophic cardiomyopathywho were judged to be at high risk for sudden death.
Results At the time of the implantation of the defibrillator,the patients were 8 to 82 years old (mean [±SD], 40±16),and 69 patients (54 percent) were less than 41 years old. Theaverage follow-up period was 3.1 years. Defibrillators wereactivated appropriately in 29 patients (23 percent), by providingdefibrillation shocks or antitachycardia pacing, with the restorationof sinus rhythm; the average age at the time of the interventionwas 41 years. The rate of appropriate defibrillator dischargewas 7 percent per year. A total of 32 patients (25 percent)had episodes of inappropriate discharges. In the group of 43patients who received defibrillators for secondary prevention(after cardiac arrest or sustained ventricular tachycardia),the devices were activated appropriately in 19 patients (11percent per year). Of 85 patients who had prophylactic implantsbecause of risk factors (i.e., for primary prevention), 10 hadappropriate interventions (5 percent per year). The intervalbetween implantation and the first appropriate discharge washighly variable but was substantially prolonged (four to nineyears) in six patients. In all 21 patients with stored electrographicdata and appropriate interventions, the interventions were triggeredby ventricular tachycardia or fibrillation.
Conclusions Ventricular tachycardia or fibrillation appearsto be the principal mechanism of sudden death in patients withhypertrophic cardiomyopathy. In high-risk patients with hypertrophiccardiomyopathy, implantable defibrillators are highly effectivein terminating such arrhythmias, indicating that these deviceshave a role in the primary and secondary prevention of suddendeath.
Source Information
From the Minneapolis Heart Institute Foundation, Minneapolis (B.J.M., A.K.A.); the Mayo Clinic, Rochester, Minn. (W.-K.S., R.F.R.); New England Medical Center and Tufts University School of Medicine, Boston (M.S.L., N.A.M.E.); the University of Alabama at Birmingham, Birmingham (A.E.E.); University of Rochester Medical Center, Rochester, N.Y. (J.P.D.); University of Washington Medical Center, Seattle (G.H.B.); Università degli Studi di Bari, Bari, Italy (S.F.); Università di Bologna, Bologna, Italy (G.B.); and Ente Ospedaliero Ospedale Galliera, Genoa, Italy (P.S.).
Address reprint requests to Dr. Maron at the Minneapolis Heart Institute Foundation, 920 E. 28th St., Suite 40, Minneapolis, MN 55407, or at gencvres{at}skypoint.com.
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Woo, A., Williams, W. G., Choi, R., Wigle, E. D., Rozenblyum, E., Fedwick, K., Siu, S., Ralph-Edwards, A., Rakowski, H.
(2005). Clinical and Echocardiographic Determinants of Long-Term Survival After Surgical Myectomy in Obstructive Hypertrophic Cardiomyopathy. Circulation
111: 2033-2041
[Abstract][Full Text]
Maron, B. J., Zipes, D. P.
(2005). Introduction: Eligibility recommendations for competitive athletes with cardiovascular abnormalities--general considerations. J Am Coll Cardiol
45: 1318-1321
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Maron, B. J., Douglas, P. S., Graham, T. P., Nishimura, R. A., Thompson, P. D.
(2005). Task Force 1: Preparticipation screening and diagnosis of cardiovascular disease in athletes. J Am Coll Cardiol
45: 1322-1326
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Maron, B. J., Ackerman, M. J., Nishimura, R. A., Pyeritz, R. E., Towbin, J. A., Udelson, J. E.
(2005). Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome. J Am Coll Cardiol
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Adabag, A. S., Casey, S. A., Kuskowski, M. A., Zenovich, A. G., Maron, B. J.
(2005). Spectrum and prognostic significance of arrhythmias on ambulatory Holter electrocardiogram in hypertrophic cardiomyopathy. J Am Coll Cardiol
45: 697-704
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Maron, B. J.
(2005). How should we screen competitive athletes for cardiovascular disease?. Eur Heart J
26: 428-430
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Kaufmann, P. A., Camici, P. G.
(2005). Myocardial Blood Flow Measurement by PET: Technical Aspects and Clinical Applications. JNM
46: 75-88
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Maron, B. J., Seidman, J.G., Seidman, C. E.
(2004). Proposal for contemporary screening strategies in families with hypertrophic cardiomyopathy. J Am Coll Cardiol
44: 2125-2132
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Maron, B. J., Dearani, J. A., Ommen, S. R., Maron, M. S., Schaff, H. V., Gersh, B. J., Nishimura, R. A.
(2004). The case for surgery in obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol
44: 2044-2053
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Hess, O. M., Sigwart, U.
(2004). New treatment strategies for hypertrophic obstructive cardiomyopathy: Alcohol ablation of the septum: the new gold standard?. J Am Coll Cardiol
44: 2054-2055
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Boriani, G., Maron, B. J., Shen, W.-K., Spirito, P.
(2004). Prevention of Sudden Death in Hypertrophic Cardiomyopathy: But Which Defibrillator for Which Patient?. Circulation
110: e438-e442
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Cohen, M. I., Rhodes, L. A., Spray, T. L., Gaynor, J. W.
(2004). Efficacy of prophylactic epicardial pacing leads in children and young adults. Ann. Thorac. Surg.
78: 197-202
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Katritsis, D. G, Camm, A.J.
(2004). Nonsustained ventricular tachycardia: where do we stand?. Eur Heart J
25: 1093-1099
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Maron, B. J., Chaitman, B. R., Ackerman, M. J., Bayes de Luna, A., Corrado, D., Crosson, J. E., Deal, B. J., Driscoll, D. J., Estes, N.A. M. III, Araujo, C. G. S., Liang, D. H., Mitten, M. J., Myerburg, R. J., Pelliccia, A., Thompson, P. D., Towbin, J. A., Van Camp, S. P., for the Working Groups of the American Heart Assoc,
(2004). Recommendations for Physical Activity and Recreational Sports Participation for Young Patients With Genetic Cardiovascular Diseases. Circulation
109: 2807-2816
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Roguin, A., Bomma, C. S., Nasir, K., Tandri, H., Tichnell, C., James, C., Rutberg, J., Crosson, J., Spevak, P. J., Berger, R. D., Halperin, H. R., Calkins, H.
(2004). Implantable Cardioverter-Defibrillators in patients with arrhythmogenic right ventricular Dysplasia/Cardiomyopathy. J Am Coll Cardiol
43: 1843-1852
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