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Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disorder, of which sudden cardiac death (SCD) is the most feared potential manifestation. Risk stratification for SCD has always been one of the greatest challenges in the management of the disease. Malignant ventricular arrhythmias are possibly the most common cause of SCD in HCM, although there are other potential causes, and in fact the pathophysiologic mechanisms leading to syncope and SCD in HCM are not completely understood. Myocyte disarray, cardiac hypertrophy, smallvessel coronary artery disease, and increased interstitial fibrosis are consistently found in the disease, and unquestionably play a role in the risk of SCD, although possibly to different and varying degrees in individual patients, depending also on the underlying genetic sarcomere alteration and tempered by additional genetic and external factors. The disease substrate contributes to functional endothelial and myocardial impairment, malignant arrhythmias, myocardial remodeling, and autonomic dysfunction, all of which can trigger sudden death under the appropriate circumstances.

Predicting risk of sudden death in hypertrophic cardiomyopathy: Can additional simple markers help?