Hypertrophic Cardiomyopathy: State of the Art.

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy. Patients with HCM who are treated according to modern guidelines can reasonably be expected to have normal longevity and good quality of life. Nevertheless, effort intolerance, heart failure, arrhythmias, and sudden cardiac death can occur in some patients. Comprehensive and longitudinal assessment with echocardiography, ambulatory electrocardiography, stress testing, and in some cases cardiac magnetic resonance imaging helps guide therapy. Symptomatic patients can be treated with medications including a new class, cardiac myosin inhibitors, developed specifically to treat the cellular pathophysiologic mechanism of HCM. For patients whose symptoms do not respond to medical therapy or in whom medical therapy is not desired, invasive options to relieve symptoms, when performed at expert centers, have robust data for successful outcomes. The goal of management is to encourage and help patients with HCM lead an active, healthy lifestyle that includes regular exercise similar to activity recommendations for the general public.