Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease and the most common cause of sudden cardiac death in young population, especially in athletes under 35 years old. Mutations occur primarily in the β-myosin heavy chain gene and involve the cardiac myosin-binding protein C gene. In this review we would like to focus on the importance of the examination of mitral valve apparatus and the mitral valve abnormalities in patients with HCM. Abnormalities in mitral valve (elongated mitral leaflets, displacement of papillary muscles, and systolic anterior motion) may be the primary pathognomonic elements, even in the absence of hypertrophy. Echocardiography is the gold standard for the diagnosis of HCM. Magnetic resonance imaging emerges as one of the most important imaging modalities for precise diagnosis, assisting in risk stratification and treatment strategy. Mitral valve abnormalities take part fundamentally in the formation of systolic anterior motion of the mitral valve and, they have substantially been repaired surgically. Although myectomy addresses the septum reduction, obstruction relief should be maximally achieved with a potential combination of myectomy and mitral valve repair.
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