Syndrome of Hypoplasia of the Right Lung and Dextroposition of the Heart: "Scimitar Sign" with Normal Pulmonary Venous Drainage

Abstract

Reported is a case in which the clinical findings of the scimitar syndrome were present but with termination of the anomalous pulmonary vein into the left atrium. There was hypoplasia of the right lung, dextroposition of the heart, and the scimitar sign in the right lower lung field. Cardiac catheterization revealed no shunt, and on pulmonary angiograms, a large common right pulmonary vein curved toward the diaphragm (as in the usual case of the scimitar syndrome), but then reversed course to enter the left atrium. No connection with the inferior vena cava was present.