Three-Dimensional Echocardiographic Evaluation of a Patient with Double-Orifice Mitral Valve, Bicuspid Aortic Valve, and Coarctation of Aorta

A 16-year-old male patient was admitted in our hospital for routine evaluation of bicuspid aortic valve and coarctation of aorta. He had a history of a subclavian patch plasty operation at 1 year of age. The maximum Doppler gradient at the coarctation site was 40 mmHg without extending into diastole (Fig. 1). In addition to these pathologies, we recognized by echocardiography that mitral valve opening was restricted (movie clip S1). Moreover, both two-dimensional (2D) and three-dimensional (3D) echocardiographic examinations (Philips iE33, Andover, MA, USA) showed division of the mitral valve into 2 symmetrical and separate valve orifices (Fig. 2; movie clips S1 and S2). There was a mild mitral regurgitation but no obstruction in left ventricular inflow and outflow. This congenital anomaly was consistent with a double-orifice mitral valve (DOMV) and it had not been detected previously in this patient. Double-orifice mitral valve is generally associated with other congenital cardiac anomalies and rarely occurs as an isolated defect. While atrioventricular septal defect is the most commonly associated lesion, it is followed by obstructive left-sided lesions and complex cyanotic heart