The selection of patients with hypertrophic cardiomyopathy (HC) for the primary prevention of sudden death (SD) with implantable cardioverter-defibrillators (ICDs) has been determined by the assessment of 5 risk factors. We examined one of these markers, the family history of HC-related SD in first-degree relatives, for which few data are available. The rate of appropriate ICD interventions was assessed in 177 consecutive patients with HC (63% men, age 45 ± 14 years) who had undergone prophylactic implantation at 2 tertiary centers, according to the identification of ≥ 1 risk markers. During a follow-up period of 4.6 ± 3 years, 25 patients (14%) had experienced appropriate ICD interventions for ventricular tachycardia/fibrillation. The patients with a risk profile that included a family history of SD experienced interventions at a similar rate (3.7/100 person-years) as the patients without a family history of SD (3.1/100 person-years, p = 0.2). The rate and frequency of appropriate ICD interventions in 42 patients who had undergone implantation solely because of a family history of SD was 2.2/100 person-years (4/42, 10%), similar to that for patients with one risk factor other than SD family history (3.4%/100 person-years; 7/50, 14%; p = 0.2) and patients with multiple risk factors with (4.5/100 person-years; 9/49, 18%) and without (3.5/100 person-years; 5/36, 14%) a family history of SD (p = 0.8). In conclusion, a family history of SD is an important risk marker in patients with HC. Patients receiving ICDs for primary prevention because of a family history of HC-related SD, whether as an isolated risk factor or combined with other markers, experienced rates of appropriate ICD discharge comparable to that of other patient subsets with increased risk.
Copyright © 2010 Elsevier Inc. All rights reserved.