Hypertrophic obstructive cardiomyopathy with abnormalities of the mitral valve complex

The mechanism of obstruction of the left ventricular outflow tract (LVOT) in hypertrophic obstructive cardiomyopathy (HOCM) is mainly due to dynamic systolic anterior motion (SAM) of the mitral valve. We report a case of HOCM with mitral regurgitation (MR) associated with complicated abnormalities of the mitral apparatus which contributed to a high pressure gradient through the LVOT. A small, 53-year-old woman was admitted for chest pain and palpitation. Examinations revealed asymmetric septal hypertrophy of the left ventricle, MR, SAM of the mitral valve and a high pressure gradient (108 mmHg) through the LVOT. Operative findings revealed an abnormally hypertrophied interventricular septum, an extensively thickened and enlarged anterior mitral leaflet (AML), malposition of the origin of the anterior papillary muscle arising closer to the aortic annulus than normal, and its direct insertion into the AML without any distinguishable chordae tendineae. The hypertrophied septum and the large and protruding AML appeared to obstruct the LVOT, resulting in a loss of subaortic clearance that was recovered after mitral valve replacement and myectomy. Pathology of the papillary muscle was characteristic of HOCM, showing disorganization and disarray of myocardial fibers, bizarre-shaped nuclei, and intercellular fibrosis, while those of the mitral leaflets negated both rheumatic changes and endocarditis.