[A case of Ellis-van Creveld syndrome with partial atrioventricular septal defect and double orifice mitral valve].

Ellis-van Creveld Syndrome (ECS) is characterized by chondrodysplasia, ectodermal dysplasia, polydactyly, and congenital cardiac defects. As congenital cardiac defects, single atrium and endocardial cusion defect are frequent in literatures. We described a case, a 68-year-old woman, presenting with ECS with partial atrioventricular septal defect and double orifice mitral valve. The partial atrioventricular septal defect was repaired by closure with a Dacron patch. The accessory orifice was located at the anterior cusp of the mitral valve, which was left, untouched, because no regurgitation had been detected preoperatively. Her postoperative course was uneventful, and the patient was discharged on 25th postoperative day.