This report presents a case of a 68-year-old woman with hypertrophic obstructive cardiomyopathy (HOCM) and concomitant mitral regurgitation (MR). Preoperative echocardiography showed stenosis of the left ventricular outflow tract (LVOT) and systolic anterior leaflet motion (SAM) of the mitral valve. She underwent mitral valve replacement( MVR) alone, and obstruction of LVOT was successfully released. Although a septal myectomy is the "gold standard" surgical therapy for HOCM, complications such as heart-block and ventricular septal perforation still remain. It was suggested that in some cases of HOCM complicated with MR, isolated MVR could be considered as the 1st-line surgical therapy.

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