Double orifice mitral valve associated with bicuspid aortic valve

A 63-year-old man with double orifice mitral valve (DOMV) and bicuspid aortic valve was reported. Preoperative echocardiography showed prolapse of the posterior leaflet and mitral regurgitation but was unable to show the existence of the duplication of the mitral valve. He underwent aortic and mitral valve replacement and did well after surgery. DOMV is a rare congenital malformation, and DOMV associated with bicuspid aortic valve is the first reported case in Japan.