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Heart. 2003 July; 89(7): 761.
PMCID: PMC1767727

Partial anomalous pulmonary venous connection or scimitar syndrome

Scimitar syndrome is a rare congenital disorder characterised by an anomalous connection of the pulmonary vein with the inferior vena cava. The anomalous vein appears as a “scimitar”-like shadow on a chest x ray and classically runs from the middle of the right lung to the cardiophrenic angle. We present a scimitar syndrome in a 77 year old woman referred for evaluation of recurrent pleuritis.

Cine magnetic resonance imaging (below left: Ao, aorta ascendens; LPA, left pulmonary artery; IVC, inferior vena cava) revealed an abnormal venous drainage of the right lung via one large pulmonary vein which drains at the inferior vena cava–right atrial junction (arrows). The right lung and airways appeared to be normal in size. The abnormal pulmonary venous drainage from the right lung was confirmed on the levophase of the right pulmonary artery angiogram (below right), which showed that the venous return from the right lung was partly directed via one anomalous vein to the inferior vena cava (arrows). The left pulmonary veins as well as the right upper lobe pulmonary vein connected and drained normally.

Partial anomalous pulmonary venous connection is usually associated with a normal or near normal life span. Many cases do not cause symptoms and are diagnosed incidentally. The natural prognosis is excellent.

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