Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy.

By B. Maron, P. Spirito, W. Shen, Tammy S. Haas, Francesco Formisano, Mark S. Link, A. Epstein, A. Almquist, J. Daubert, T. Lawrenz and 12 more. Journal of the American Medical Association (JAMA), 2007

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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.

Subfields: Cardiology and Cardiovascular Medicine

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In Cardiology and Cardiovascular Medicine

Citations in the corpus

Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. (2014, background): “Although multiple risk factors in a given patient intuitively convey the greatest SD risk, there is compelling evidence that a single strong marker within the clinical profile of an individual patient (particularly in those <60 years of age) can be sufficient evidence to raise the option of a primar”
ACC/AHA/HRS 2008 Guidelines for device-based therapy of cardiac rhythm abnormalities. (2008, background): “Recent analyses indicate that in a high-risk HCM cohort, ICD interventions were frequent and were highly effective in restoring normal sinus rhythm (245).”