Three-dimensional computed tomography showing partial anomalous pulmonary venous connection complicated by the scimitar syndrome.

The patient is a 41-year-old woman who was referred to our hospital because of an abnormal chest radiograph. On admission, she was asymptomatic, but her cardiac silhouette was shifted to the right on chest radiograph. From contrast-enhanced chest computed tomography (CT), we suspected that the right upper pulmonary vein connected to the inferior vena cava. The CT also revealed hypoplasia of the right lung. The 3-dimensional imaging reconstructed from the CT clearly showed the right upper pulmonary vein connected to the inferior vena cava (Figure). During cardiac catheterization, oxymetry sampling showed a step up of the blood oxygen content at the inferior vena cava level just below the border of the right atrium, which indicated left-to-right shunting at this level. In addition, the upper pulmonary venous drainage into the inferior vena cava was also shown by pulmonary arteriography. This partial anomalous pulmonary venous connection associated with hypoplasia of the right lung and dextroposition of the heart is designated Scimitar Syndrome. Left-to-right shunt was slight and pulmonary hypertension was not present. Consequently, no surgical treatment was required.