Scimitar syndrome: the curved Turkish sabre

Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies characterized by an anomalous pulmonary vein (scimitar vein) that drains into the inferior vena cava, a hypogenetic right lung, and dextroposition of the heart. It has been reported in 3% to 6% of patients with partial anomalous pulmonary venous connection. Patients are either diagnosed early with severe symptoms (infantile type) or late with minimal symptoms (childhood/adult type). In this chapter we reviewed the history, pathophysiology, presentation, diagnosis, and treatment methods of Scimitar syndrome in the current era.