A case of DOMV associated with incomplete ECD in a 17-year old girl is presented. Preoperatively, the mitral valve was suspected to have two orifice divided by the bridging tissue with sector echocardiography. DOMV is very rare congenital malformation, and 25 cases of DOMV are reported in Japan. Two of them were diagnosed as DOMV preoperatively with sector echocardiography. Sector echocardiography is useful to diagnose DOMV and to know the shape of it preoperatively.
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