Radiographic Features of the Scimitar Syndrome

Publisher Website
Google Scholar
Abstract
The scimitar syndrome always involves the right lung and its vascular supply. It consists of hypo-plasia of the right pulmonary artery and right lung with consequent dextroposition of the heart, anomalous arterial supply of the right lower lobe by a systemic artery from the abdominal aorta, and anomalous venous drainage of the right lung. The anomalous vein has a characteristic scimitar-like shape and enters the inferior vena cava at the level of the diaphragm. The anomalous vein was clearly seen on postero-anterior roentgenograms in all 7 cases studied. Hypoplasia of the right lung varies in degree. Patients with marked hypoplasia were prone to repeated respiratory infections, and the development of pleural and atelectatic changes caused indistinctness of the right heart border. The syndrome may be associated with additional intracardiac defects as observed in 3 of our patients. Lami-nagraphy and venous angiography allow a better demonstration of the anomalous vein, but the procedure of choice is selective pulmonary arteriography which usually also demonstrates the anomalous arterial supply to the right lower lobe.