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2f-1. Anomalous pulmonary venous connection (Scimitar sign). (Legend.)

A. Anomalous right pulmonary vein is adjacent to and parallels the right atrium.

B. Left anterior oblique view shows the anomalous vein ending at an area of increased density where it enters the right atrium.

Scimitar syndrome

Scimitar syndrome comprises PAPVC when the right lung drains anomalously by a single trunk to the inferior vena cava at the level of the diaphragm, in association with hypoplasia of the ipsilateral pulmonary artery, hypoplasia of the ipsilateral lung and abberant systemic arterial supply to the ipsilateral lung (aorto-pulmonary collateral). Dextrocardia is often associated with the syndrome. Scimitar is a "curved sword" describing the outline of the anomalous venous channel. This was first described by Neill et al. in 1960.