Management of Hypertrophic Cardiomyopathy: JACC State-of-the-Art Review

J Am Coll Cardiol. 2022 Feb 1;79(4):390-414. doi: 10.1016/j.jacc.2021.11.021.


Hypertrophic cardiomyopathy (HCM), a relatively common, globally distributed, and often inherited primary cardiac disease, has now transformed into a contemporary highly treatable condition with effective options that alter natural history along specific personalized adverse pathways at all ages. HCM patients with disease-related complications benefit from: matured risk stratification in which major markers reliably select patients for prophylactic defibrillators and prevention of arrhythmic sudden death; low risk to high benefit surgical myectomy (with percutaneous alcohol ablation a selective alternative) that reverses progressive heart failure caused by outflow obstruction; anticoagulation prophylaxis that prevents atrial fibrillation-related embolic stroke and ablation techniques that decrease the frequency of paroxysmal episodes; and occasionally, heart transplant for end-stage nonobstructive patients. Those innovations have substantially improved outcomes by significantly reducing morbidity and HCM-related mortality to 0.5%/y. Palliative pharmacological strategies with currently available negative inotropic drugs can control symptoms over the short-term in some patients, but generally do not alter long-term clinical course. Notably, a substantial proportion of HCM patients (largely those identified without outflow obstruction) experience a stable/benign course without major interventions. The expert panel has critically appraised all available data and presented management insights and recommendations with concise principles for clinical decision-making.

Keywords: atrial fibrillation; defibrillation; heart failure; hypertrophic cardiomyopathy; stroke; sudden death; surgery.

Publication types

  • Systematic Review

MeSH terms

  • Cardiomyopathy, Hypertrophic / complications
  • Cardiomyopathy, Hypertrophic / mortality
  • Cardiomyopathy, Hypertrophic / therapy*
  • Death, Sudden, Cardiac / prevention & control
  • Humans