Findings: This PA view of the chest demonstrates a hypogenetic right lung with a small right hemithorax and shift of the heart and mediastinum to the right. In addition, there is a large anomalous pulmonary vein draining into the IVC.
Diagnosis: Scimitar syndrome.
Discussion: Scimitar syndrome represents one manifestation of a more generalized disorder called congenital pulmonary venolobar syndrome. This syndrome encompasses a number of congenital abnormalities including: hypogenetic lung, partial anomalous pulmonary venous return, absence of pulmonary arteries, pulmonary sequestration, absence of IVC, and accessory diaphragm. When hypogenetic lung and partial anomalous pulmonary venous return coexist, it is termed scimitar syndrome. The anomalous draining pulmonary vein most commonly drains into the IVC, but may on occasion drain into the hepatic veins, portal veins, azygous vein, coronary sinus, or right atrium. The abnormality is almost always right sided. Associated anomalies include branchial isomerism and anomalous arterial supply to the hypogenetic lung (usually systemic arteries such as bronchial). In most cases the anomalous draining vein is demonstrated roentgenologically as a broad curving opacity just to the right of the right heart border. This in association with a small right hemithorax/lung is diagnostic of scimitar syndrome.
References:
Woodring JH. Congenital Pulmonary Venolobar Syndrome.
Revisited Radiographics 1994;14:349-369.
Fraser, R. Synopsis of Diseases of the Chest. W. B. Saunders,
Philadelphia. 1994:277.
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