Clinical and Echocardiographic Characteristics of Scimitar Syndrome

Seven patients with scimitar syndrome underwent retrospective clinical and echocardiographic examination. The findings were: respiratory infection with dyspnea on moderate exercise in 90%, scimitar sign in 4 (57%), dextrocardia in 5 (71%), and atrial septal defect in five (71%), one of whom had patent ductus arteriosus. Overall, 2 patients had patent ductus arteriosus: one also had aortic coarctation and the other, a bicuspid aortic valve. Dilation of the right cavities was found in 5 (71%) and blunt edge in 5 (71%). In 2 patients, anomalous drainage was into the right atrium; in another 2, into the inferior vena cava; and in 3, towards the junction of the right atrium and the inferior vena cava. In 3 patients, drainage was obstructed. Six patients with cardiac abnormalities proceeded to surgery. Scimitar syndrome is a rare entity. In the large majority of patients (86%), diagnosis and follow-up can be performed echocardiographically.