Abstract

Background —Death resulting from hypertrophic cardiomyopathy (HCM), particularly when sudden, has been reported to be largely confined to young persons. These data emanated from tertiary HCM centers with highly selected referral patterns skewed toward high-risk patients. Methods and Results —The present analysis was undertaken in an international population of 744 consecutively enrolled and largely unselected patients more representative of the overall HCM spectrum. HCM-related death occurred in 86 patients (12%) over 8±7 years (mean±SD). Three distinctive modes of death were as follows: (1) sudden and unexpected (51%; age, 45±20 years); (2) progressive heart failure (36%; age, 56±19 years); and (3) HCM-related stroke associated with atrial fibrillation (13%; age, 73±14 years). Sudden death was most common in young patients, whereas heart failure– and stroke-related deaths occurred more frequently in midlife and beyond. However, neither sudden nor heart failure–related death showed a statistically significant, disproportionate age distribution ( P =0.06 and 0.5, respectively). Stroke-related deaths did occur disproportionately in older patients ( P =0.002). Of the 45 patients who died suddenly, most (71%) had no or mild symptoms, and 7 (16%) participated in moderate to severe physical activities at the time of death. Conclusions —HCM-related cardiovascular death occurred suddenly, or as a result of heart failure or stroke, largely during different phases of life in a prospectively assembled, regionally based, and predominantly unselected patient cohort. Although most sudden deaths occurred in adolescents and young adults, such catastrophes were not confined to patients of these ages and extended to later phases of life. This revised clinical profile suggests that generally held epidemiological tenants for HCM have been influenced considerably by skewed reporting from highly selected populations. These data are likely to importantly affect risk stratification and treatment strategies importantly for the prevention of sudden death in HCM.