Population Burden of Sudden Death Associated With Hypertrophic Cardiomyopathy
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Hypertrophic cardiomyopathy (HCM) is a genetically transmitted cardiac disease, characterized by increased left ventricular (LV) wall thickness in the absence of abnormal loading conditions.1 The estimated prevalence of HCM is ≈1 per 500 in the general population,2 with a diverse clinical course including heart failure and sudden cardiac arrest (SCA), but also asymptomatic survival to normal life expectancy.3 SCA attributable to ventricular arrhythmias is a major cause of mortality in young and middle-aged patients with HCM.4 With modern management strategies, including implantable cardioverter-defibrillator therapy for high-risk individuals, contemporary disease-related mortality has been reported to be ≈0.5% annually.4 These estimates are derived from several HCM patient registries, representing a somewhat selected patient population. However, the absence of symptoms causes HCM to remain undetected in a large proportion of patients. Because few data exist on the burden of HCM-related SCA in the community, in the present study we assessed the incidence and characteristics of HCM-related SCA in the young and middle-aged general population from the ongoing Oregon SUDS (Oregon Sudden Unexpected Death Study). This study was approved by the institutional review boards of Cedars-Sinai Medical Center, Oregon Health …