Purpose of review: To briefly review the pathophysiology and natural history of hypertrophic cardiomyopathy (HCM) and to describe the diagnosis, assessment, and contemporary management strategies.
Recent findings: HCM-related mortality remains low; however, symptoms due in large part to LVOT obstruction remain a clinical dilemma. Several medical therapies have been shown to reduce symptoms and improve functional capacity, including several recent phase 2 clinical trials involving the novel myosin modulator mavacamten. In patients with refractory symptoms, septal reduction therapy or advanced therapies remain viable options in many cases. HCM is a complex and heterogeneous disease with diverse presentations and variable anatomy and clinical outcomes. The majority of patients will remain asymptomatic or with minimal symptoms and long-term survival remains high. For symptomatic patients, a variety of medical therapies, along with septal reduction therapies, have been shown to reduce symptoms and improve functional capacity.
Keywords: Clinical heterogeneity; Inherited cardiomyopathy; Left ventricular hypertrophy; Left ventricular outflow obstruction; Septal reduction therapy; Sudden cardiac death.