Ellis-Van Creveld Syndrome with Dual Coronary Sinus Ostiae: A Case Report

Background: Ellis–van Creveld syndrome (EVCS) is a rare disease characterized by various cardiac and skeletal abnormalities. It carries a high mortality in early life and accounts for 50% mortality during infancy. There are only limited numbers of cases reported worldwide. Case presentation: A 9-years-old girl presented with dyspnoea on exertion of 4 months duration (NYHA II). She was short statured and had bilateral upper limb polydactyly, dysplastic teeth. Cognitive and motor developments were normal. On evaluation, 2D Echocardiography showed common atrium, partial AV canal defect, and mild tricuspid regurgitation. Intraoperatively in addition to above findings the patient also had dual coronary sinus ostiae and persistent left superior vena cava (LSVC) draining into the left atrial roof. The patient underwent successful correction of associated pathologies (Intracardiac repair). The perioperative period was uneventful. Conclusion: This case is being reported to emphasize the rare occurrence of Ellis–van Creveld syndrome and even rare association of dual coronary sinus ostiae with it.