NDLI: Implantable Cardioverter-Defibrillators and Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy

Content Provider	JAMA Network
Author	Maron, Barry J. Spirito, Paolo Shen, Win-Kuang Haas, Tammy S. Formisano, Francesco Link, Mark S. Epstein, Andrew E. Almquist, Adrian K. Daubert, James P. Lawrenz, Thorsten Boriani, Giuseppe Estes, N. A. Mark Favale, Stefano Piccininno, Marco Winters, Stephen L. Santini, Massimo Betocchi, Sandro Arribas, Fernando Sherrid, Mark V. Buja, Gianfranco Semsarian, Christopher Bruzzi, Paolo
Copyright Year	2007
Abstract	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.
Ending Page	412
Starting Page	405
Page Count	8
File Format	PDF HTM / HTML
ISSN	00987484
DOI	10.1001/jama.298.4.405
Issue Number	4
Journal	JAMA
Volume Number	298
Language	English
Publisher	American Medical Association
Publisher Date	2007-07-25
Access Restriction	Open
Subject Keyword	hypertrophic cardiomyopathy sudden death sudden cardiac death implantable defibrillators follow-up ventricular tachycardia implantable defibrillator insertion left ventricular hypertrophy non-sustained ventricular tachycardia cardiac arrest syncope primary prevention body substance discharge cardiac arrhythmia secondary prevention
Content Type	Text
Resource Type	Article
Subject	Medicine

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