医学
酒精间隔消融
心房颤动
自然史
重症监护医学
心肌病
心力衰竭
导管消融
心脏移植
肥厚性心肌病
疾病
心脏病
变向性
内科学
心房颤动的处理
心源性猝死
心脏病学
梗阻性心肌病
作者
Barry J. Maron,Milind Y. Desai,Rick A. Nishimura,Paolo Spirito,Harry Rakowski,Jeffrey A. Towbin,Joseph A. Dearani,Ethan J. Rowin,Martin S. Maron,Mark V. Sherrid
标识
DOI:10.1016/j.jacc.2021.11.021
摘要
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.
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