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Implantable Cardioverter-Defibrillators and Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy
Barry J. Maron, MD;
Paolo Spirito, MD;
Win-Kuang Shen, MD;
Tammy S. Haas, RN;
Francesco Formisano, MD;
Mark S. Link, MD;
Andrew E. Epstein, MD;
Adrian K. Almquist, MD;
James P. Daubert, MD;
Thorsten Lawrenz, MD;
Giuseppe Boriani, MD, PhD;
N. A. Mark Estes III, MD;
Stefano Favale, MD;
Marco Piccininno, MD;
Stephen L. Winters, MD;
Massimo Santini, MD;
Sandro Betocchi, MD;
Fernando Arribas, MD;
Mark V. Sherrid, MD;
Gianfranco Buja, MD;
Christopher Semsarian, MB, BS, PhD;
Paolo Bruzzi, MD, PhD
JAMA. 2007;298:405-412.
Context Recently, the implantable cardioverter-defibrillator (ICD) has been promoted for prevention of sudden death in hypertrophic cardiomyopathy (HCM). However, the effectiveness and appropriate selection of patients for this therapy is incompletely resolved.
Objective To study the relationship between clinical risk profile and incidence and efficacy of ICD intervention in HCM.
Design, Setting, and Patients Multicenter registry study of ICDs implanted between 1986 and 2003 in 506 unrelated patients with HCM. Patients were judged to be at high risk for sudden death; had received ICDs; underwent evaluation at 42 referral and nonreferral institutions in the United States, Europe, and Australia; and had a mean follow-up of 3.7 (SD, 2.8) years. Measured risk factors for sudden death included family history of sudden death, massive left ventricular hypertrophy, nonsustained ventricular tachycardia on Holter monitoring, and unexplained prior syncope.
Main Outcome Measure Appropriate ICD intervention terminating ventricular tachycardia or fibrillation.
Results The 506 patients were predominately young (mean age, 42 [SD, 17] years) at implantation, and most (439 [87%]) had no or only mildly limiting symptoms. ICD interventions appropriately terminated ventricular tachycardia/fibrillation in 103 patients (20%). Intervention rates were 10.6% per year for secondary prevention after cardiac arrest (5-year cumulative probability, 39% [SD, 5%]), and 3.6% per year for primary prevention (5-year probability, 17% [SD, 2%]). Time to first appropriate discharge was up to 10 years, with a 27% (SD, 7%) probability 5 years or more after implantation. For primary prevention, 18 of the 51 patients with appropriate ICD interventions (35%) had undergone implantation for only a single risk factor; likelihood of appropriate discharge was similar in patients with 1, 2, or 3 or more risk markers (3.83, 2.65, and 4.82 per 100 person-years, respectively; P = .77). The single sudden death due to an arrhythmia (in the absence of advanced heart failure) resulted from ICD malfunction. ICD complications included inappropriate shocks in 136 patients (27%).
Conclusions In a high-risk HCM cohort, ICD interventions for life-threatening ventricular tachyarrhythmias were frequent and highly effective in restoring normal rhythm. An important proportion of ICD discharges occurred in primary prevention patients who had undergone implantation for a single risk factor. Therefore, a single marker of high risk for sudden death may be sufficient to justify consideration for prophylactic defibrillator implantation in selected patients with HCM.
Author Affiliations: Hypertrophic Cardiomyopathy Center of the Minneapolis Heart Institute Foundation, Minneapolis, Minnesota (Drs Maron and Almquist and Ms Haas); Ente Ospedaliero Ospedali Galliera, Genoa, Italy (Drs Spirito, Formisano, and Piccininno); Mayo Clinic, Rochester, Minnesota (Dr Shen); New England Medical Center and Tufts University School of Medicine, Boston, Massachusetts (Drs Link and Estes); University of Alabama at Birmingham (Dr Epstein); University of Rochester Medical Center, Rochester, New York (Dr Daubert); Academic Teaching Hospital of University of Muenster, Bielefeld, Germany (Dr Lawrenz); Universitá di Bologna, Bologna, Italy (Dr Boriani); Universitá degli Studi di Bari, Bari, Italy (Dr Favale); Morristown Memorial Hospital, Morristown, New Jersey (Dr Winters); Ospedale S. Filippo Neri, Rome, Italy (Dr Santini); Università Federico II, Naples, Italy (Dr Betocchi); Hospital 12 de Octubre, Madrid, Spain (Dr Arribas); St Lukes-Roosevelt Hospital Center, New York, New York (Dr Sherrid); University of Padua, Padua, Italy (Dr Buja); Department of Cardiology, Royal Prince Alfred Hospital and Centenary Institute, Sydney, Australia (Dr Semsarian); and Istituto Nazionale per la Ricerca sul Cancro, Genoa, Italy (Dr Bruzzi).
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