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Efficacy of Implantable Cardioverter–Defibrillators for the Prevention of Sudden Death in Patients with Hypertrophic Cardiomyopathy

Maron, Barry J., Shen, Win-Kuang, Link, Mark S., Epstein, Andrew E., Almquist, Adrian K., Daubert, James P., Bardy, Gust H., Favale, Stefano, Rea, Robert F., Boriani, Giuseppe, Estes, N.A. Mark, Spirito, Paolo, Casey, Susan A., Stanton, Marshall S., Betocchi, Sandro·2000·New England Journal of Medicine
DOI 10.1056/nejm200002103420601
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Abstract

BACKGROUND: Hypertrophic cardiomyopathy is a genetic disease associated with a risk of ventricular tachyarrhythmias and sudden death, especially in young patients. METHODS: We conducted a retrospective multicenter study of the efficacy of implantable cardioverter-defibrillators in preventing sudden death in 128 patients with hypertrophic cardiomyopathy who 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. The average follow-up period was 3.1 years. Defibrillators were activated appropriately in 29 patients (23 percent), by providing defibrillation shocks or antitachycardia pacing, with the restoration of sinus rhythm; the average age at the time of the intervention was 41 years. The rate of appropriate defibrillator discharge was 7 percent per year. A total of 32 patients (25 percent) had episodes of inappropriate discharges. In the group of 43 patients who received defibrillators for secondary prevention (after cardiac arrest or sustained ventricular tachycardia), the devices were activated appropriately in 19 patients (11 percent per year). Of 85 patients who had prophylactic implants because of risk factors (i.e., for primary prevention), 10 had appropriate interventions (5 percent per year). The interval between implantation and the first appropriate discharge was highly variable but was substa…

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Crossref · 10.1056/nejm200002103420601

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1 of 1 references verified0 citation errors · 0 not checkable

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  • Efficacy of the implantable cardioverter-defibrillator for primary prevention in patients with hypertrophic cardiomyopathy was first reported in 2000.

    “Efficacy of the implantable cardioverter-defibrillator (ICD) for primary prevention in patients with hypertrophic cardiomyopathy was first reported in 2000”

    From Current management of hypertrophic cardiomyopathy (2025) · cites this paper as [94]

Citations

Cites

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Metadata

Authors
Maron, B. J., Shen, W.-K., Link, M. S., Epstein, A. E., Almquist, A. K., Daubert, J. P., Bardy, G. H., Favale, S., Rea, R. F., Boriani, G., Estes, N. A. M., Spirito, P., Casey, S. A., Stanton, M. S., & Betocchi, S.
Venue
New England Journal of Medicine
Publisher
Massachusetts Medical Society
Published
2000-02-10
Volume
342
Issue
6
Pages
365-373
ISSN
  • 0028-4793
  • 1533-4406
DOI
10.1056/nejm200002103420601
Cited by
832
References
1
Language
en

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