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What are the current best practices for diagnosing, risk-stratifying, and managing hypertrophic cardiomyopathy?
Hypertrophic cardiomyopathy (HCM) is a complex heterogenous disorder that is defined by left ventricular hypertrophy without abnormal loading conditions. Left ventricular ejection fraction is supranormal and diastolic dysfunction slows ventricular filling. Clinical presentation is highly variable.
HCM is the most common monogenic cardiac disorder. Affected genes encode sarcomeric proteins.
Cardiac imaging — particularly echocardiography and cardiac MRI — plays a central role in diagnosis and risk assessment.
Annual incidence of sudden cardiac death in patients with high-risk HCM is less than 0.5% with the use of impla…