Scimitar syndrome associated with ventricular septal defect and left pulmonary vein stenosis

Scimitar syndrome is a rare congenital cardiac anomaly, which is characterized by anomalous right pulmonary venous return to the inferior vena cava. In this article, we report a six-month-old girl with the diagnosis of Scimitar syndrome associated with ventricular septal defect, left pulmonary venous stenosis and severe pulmonary hypertension. The patient underwent a successful surgical treatment of all pathologies at a single session. Scimitar syndrome is a rare congenital cardiac anomaly characterized by anomalous pulmonary venous return of the right lung to the inferior vena cava (IVC). The anomaly is usually associated with right lung hypoplasia with dextroposition of the heart and abnormal arterial supply of a variable degree to the right lung. [1-4] The presence of additional cardiac anomalies generally aggravates the clinical picture by causing significant left-to-right shunt, congestive heart failure, and severe pulmonary hypertension. Herein, we present a patient with scimitar syndrome associated with ventricular septal defect (VSD), left pulmonary venous stenosis (PVS), right pulmonary arterial stenosis (PAS), and abnormal arterial supply from the abdominal aorta to the right lung.