Double orifice mitral valve associated with endocardial cushion defect

Duplication of the mitral valve is a rare congenital cardiac anomaly. We encountered a case of duplication of the mitral valve associated with a partial form of endocardial cushion defect in a 6-year-old girl. The mitral orifice was separated by a fibrous tissue and each orifice provided papillary muscle and complete subvalvular mechanisms except for the cleft region. The fibrous tissue also provided a subvalvular apparatus. The cleft was repaired without complication. The short axis view of the two-dimensional echocardiogram demonstrated two separate holes in the mitral valve, which constituted the most obvious diagnostic sign prior to surgery.