[Scimitar syndrome].

80 Background --Scimitar syndrome, which presents with an anomalous right pulmonary vein that drains part or the entire right lung into the inferior vena cava (IVC) as its hallmark, is a rare association of congenital cardiopulmonary anomalies with an incidence of approximately 1 to 3 per 100,000 live births worldwide. It commonly presents with symptoms of respiratory distress and has an over-all mortality rate of 45%. Case --We present a case of a two month old male infant who presented with difficulty of breathing, for which he was mechanically ventilated. Chest radiograph showed dextroposed heart. Further work-ups, which included echocardiography, high resolution CT scan and hemodynamic studies, demonstrated the presence of an anomalous right pulmonary vein that drains into the inferior vena cava. Patient underwent ligation of the feeding vessels, which he tolerated well. Conclusion --This case illustrates the value of recognizing scimitar syndrome as one of the differentials in children who presents with respiratory distress. Although it is rare, timely surgical intervention improves the outcome of these patients. Phil Heart Center J 2008; 14(1):80-83.