The isolated congenital mitral regurgitation (MR) is a rare anomaly. We have successfully treated a 7-year-old boy who had MR due to a partial defect of the anterior leaflet. Echocardiogram revealed moderate MR at the anterior leaflet as a result of its partial defect. Another MR was also detected at the coaptation zone of the 2 leaflets due to mild prolapse of the anterior leaflet. The mitral valve was approached via atrial septostomy and a partial defect (8 x 6 mm) was found near the postero-medial commissure of the anterior leaflet. Any chordae did not develop around the defect or subvalvular lesions. Other parts of the leaflet was seemed normal and any inflammatory or degenerative changes were not detected in the rest of the leaflets. We have done direct closure of the defect of the leaflet and also added annuloplasty at the posteromedial commissure by modified Kay method.
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