RESUMO
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.
Assuntos
Cardiomiopatia Hipertrófica , Cardiomiopatia Hipertrófica/terapia , HumanosAssuntos
Fibrilação Atrial , Cardiomiopatia Hipertrófica , Humanos , Fibrilação Atrial/diagnóstico , Fibrilação Atrial/cirurgia , Qualidade de Vida , Antiarrítmicos/uso terapêutico , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/diagnóstico , Cardiomiopatia Hipertrófica/terapiaRESUMO
Hypertrophic cardiomyopathy is one of the most common inherited cardiovascular diseases and is characterized by a heterogeneous appearance and natural history. Although previously thought to be a disease of the young, HCM is frequently diagnosed in patients over age 50. A careful history and physical examination and readily available non-invasive testing will diagnose most cases, but genetic testing can identify those not expressing the typical phenotype. Treatment of symptomatic patients is targeted toward improving LV diastolic function; for patients with the obstructive form HCM, treatment involves relieving outflow tract obstruction. Identification of those at risk for sudden death may require consideration of prophylactic defibrillator placement.