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The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.

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

Open access
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·2007·JAMA
DOI 10.1001/jama.298.4.405
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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.

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Metadata

Authors
Maron, B. J., Spirito, P., Shen, W.-K., Haas, T. S., Formisano, F., Link, M. S., Epstein, A. E., Almquist, A. K., Daubert, J. P., Lawrenz, T., Boriani, G., Estes, N. A. M., Favale, S., Piccininno, M., Winters, S. L., Santini, M., Betocchi, S., Arribas, F., Sherrid, M. V., Buja, G., Semsarian, C., & Bruzzi, P.
Venue
JAMA
Publisher
American Medical Association (AMA)
Published
2007-07-25
Volume
298
Issue
4
Pages
405-12
ISSN
  • 0002-9955 (ISSN-L)
  • 0098-7484
DOI
10.1001/jama.298.4.405
OpenAlex
W2127235450
Cited by
804
References
3
FWCI
40.18
Type
journal article
Language
en
Open access
green

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