| Summary: | Double orifice mitral valve (DOMV), is an uncommon anomaly characterized by a mitral valve with a single fibrous annulus with two orifices opening into the left ventricle. Subvalvular structures, especially the tensor apparatus, invariably shows various degrees of abnormalities. DOMV may usually allow normal blood flow between the left atrium and left ventricle but can sometimes substantially obstruct mitral valve inflow or produce mitral valve regurgitation. Recognition of DOMV and awareness of the anatomic variations are important to achieve good therapeutic results. Treatment is only necessary if significant mitral stenosis or mitral regurgitation is present. Here, we present a rare case of DOMV with ventricular septal defect which was detected by two-dimensional echocardiography.
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