Congenital double-orifice mitral valve is a rare congenital heart disease and causes mitral-valve regurgitation and/or stenosis. We present a case of congenital double-orifice mitral valve with mitral regurgitation that was not verified by preoperative echocardiography. It was confirmed by open-heart surgery and was successfully repaired with comprehensive techniques. It is possible to achieve better long-term results and life quality of congenital double-orifice mitral valve with valve repair.
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