The mitral valve in hypertrophic cardiomyopathy: Old versus new concepts

Elongation and pathological thickening of the mitral valve (MV) is commonly seen in hypertrophic cardiomyopathy (HCM), and its pathogenic basis is poorly understood. Associated features include mal-positioning of the papillary muscles and MV, as well as systolic anterior motion (SAM) of the MV leaflets, which can worsen the turbulence and dynamic left ventricular outflow tract (LVOT) gradient. Coaptation of the MV leaflets depends on both anterior and posterior leaflet length and position, and failure of either to optimally adapt in this setting can result in mitral regurgitation or worsened LVOT obstruction. The cause of MV enlargement in HCM is not currently understood, and several different hypotheses may be relevant. The lack of correlation between MV size and the severity of left ventricular hypertrophy, as well as the early findings in genetically predisposed individuals with sarcomere mutations, suggest that it may be an intrinsic aspect of HCM in certain individuals. Other evidence points to a reactive process in the setting of excess production of paracrine growth factors in diseased myocardium that may influence valve overgrowth. Improved understanding of the responsible adaptive mechanisms will pave the way for studies targeted on the prevention and treatment of MV disease in HCM.