Evolution of risk stratification and sudden death prevention in hypertrophic cardiomyopathy: Twenty years with the implantable cardioverter-defibrillator

摘要

Hypertrophic cardiomyopathy (HCM) is a relatively common inherited myocardial disorder, once regarded as largely untreatable with ominous prognosis and most visibly as a common cause of sudden death (SD) in the young. Over the last several years, HCM has been transformed into a contemporary treatable disease with management options that significantly alter clinical course. With the use of implantable cardioverter-defibrillators (ICDs) in the HCM patient population, a new paradigm has emerged, with primary prevention device therapy reliably terminating potentially lethal ventricular tachyarrhythmias (3%-4% per year) and being largely responsible for a >10-fold decrease in disease-related mortality (to 0.5% per year), independent of age.