This review summarizes 50 years of insights into the electrophysiological substrate, risk markers, and the evolving role of ICDs for preventing sudden death in patients with hypertrophic cardiomyopathy.
A fter 50 years of recognition and study, it is evident that hypertrophic cardiomyopathy (HCM) is a particularly heterogeneous and unpredictable disease with respect to its clinical expression and natural history.12345 Sudden death (SD) continues to be the most devastating complication of HCM, dating from its modern description.6 However, there were virtually no effective strategies for SD prevention until recently when HCM entered the implantable cardioverterdefibrillator (ICD) era, 7-9 creating an enhanced focus on risk stratification and reliable identification of high-risk patients.8 -14 Consequently, it is timely to summarize what has been learned about HCM-related SD over these 5 decades, including the electrophysiological substrate, epidemiology, risk markers, and ultimately the role of ICDs, which have changed the natural course of this complex disease.This discussion emphasizes the clarification of areas in which disagreement and divergent views arise, by using available information to achieve a balanced assessment of SD in HCM.However, these observations ultimately represent only a "snapshot" in time for what undoubtedly will prove to be an evolving area of investigation and understanding.
Barry J. Maron (2010) studied this question.