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Scimitar syndrome, or pulmanory venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung.[1] This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as The term Scimitar syndrome arises from the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava.[2] This radiographic density often has the shape of a scimitar, a type of curved sword.[2]
The anomalous venous return forms a curved shadow on chest x-ray such that it resembles a scimitar. This is called the Scimitar Sign. Associated abnormalities include right lung hypoplasia with associated dextroposition of the heart, pulmonary artery hypoplasia and pulmonary sequestration.
The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, and more recently by CT angiography or MR Angiography.
Surgical correction should be considered in the presence of significant left to right shunting (Qp:Qs = 2:1) and pulmonary hypertension. This involves creation of an inter-atrial baffle to redirect the pulmonary venous return into the left atrium. Alternatively, the anomalous vein can be re-implanted directly into the left atrium.
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