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The Present and Future
JACC State-of-the-Art Review
Management of Hypertrophic Cardiomyopathy: JACC State-of-the-Art ReviewUnder a Creative Commons license
Open access
Highlights
•
Contemporary treatments have effectively transformed HCM into a highly treatable condition with relatively low morbidity/mortality.
•Sudden death in patients with HCM can be prevented through risk stratification and use of ICDs.
•Surgical myectomy has become a highly effective, low-risk operation in experienced centers to relieve outflow tract obstruction and symptoms.
Abstract
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.
Key Words
atrial fibrillation
defibrillation
heart failure
hypertrophic cardiomyopathy
stroke
sudden death
surgery
Abbreviations and Acronyms
ASA
alcohol septal ablation
HCM
hypertrophic cardiomyopathy
ICD
implantable cardioverter-defibrillator
LGE
late gadolinium enhancement
NYHA
New York Heart Association
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© 2022 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation.