Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM), a genetic sarcomeric disorder associated with myocyte disarray and scar deposition, is intimately linked to sudden cardiac death (SCD) due to malignant ventricular arrhythmias. HCM is the most common inherited cardiomyopathy, affecting between two and 20 adults per 1,000. Excluding alternative causes of hypertrophy of the left ventricle is fundamental to making a diagnosis of HCM and is often clinically challenging.

With the advent of ICDs and early detection of the disease, modern cohorts have mortality rates <1 %. SCD due to sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) is still the most common cause of mortality, accounting for half of all HCM-related deaths. Sudden death in HCM is one of the leading causes of death in individuals under the age of 40. While there appears to be no difference in SCD rates based on gender, age is an important factor: SCD is more common in younger patients, especially those <35 years; however, up to one-fifth of SCDs have been reported to occur in patients >65 years. Identifying patients within this heterogeneous disorder who are at high risk of sudden death is a challenge.