• Michel Desnos.
• Hôpital Européen Georges Pompidou, 20, rue Leblanc - 75015 Paris. michel.desnos@egp.aphp.fr
• B Acad Nat Med Paris. 2012 Apr 1;196(4-5):997-1009; discussion 1009-10.

AbstractHypertrophic cardiomyopathy (HCM) is a myocardial disorder characterized by left ventricular hypertrophy with no apparent cause (such as severe hypertension, aortic valve stenosis, etc.). The clinical diagnosis is based on cardiac imaging, commonly using 2D echocardiography and increasingly CMR. HCM is the leading cause of sudden death in young people, especially on the sports field. Many patients remain asymptomatic throughout life, while others develop heart failure, atrial fibrillation and stroke. HCM is the most common genetic (autosomal dominant) cardiovascular disease, with variable penetrance and expression. It is caused by mutations in genes coding for cardiac sarcomeric proteins. Genetic counseling and clinical risk stratification are crucial for all patients. Medical treatment with B-blockers or verapamil improves symptoms but has not been show to modify the clinical course. Patients with outflow obstruction and severe symptoms unresponsive to medical therapy are candidates for alcohol septal ablation or surgical myectomy. Current approaches focus on the prevention of sudden death by means of implantable defibrillators in high-risk patients.

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