[Cardiomyopathies. VIII. Sudden death in hypertrophic cardiomyopathy].

The natural history of patients with hypertrophic cardiomyopathy (HCM) may be shadowed by the appearance of sudden death (SD). The identification and management of patients with HCM who are at increased risk of SD remains a major problem and a clinical challenge. Several mechanisms have been implicated in the pathogenesis of the disease, its symptomatic status and prognosis. However, the definitive cause of SD in HCM is rarely ascertainable. Genetic factors are emerging as new and important determinants of life expectancy among affected individuals. Conversely, ventricular arrhythmias remain the most useful single factor in the prediction of SD. In this review we highlight the assessment of patients at high risk, potential mechanisms of SD and major unanswered issues concerning prevention and treatment.