Uncommon variants of the scimitar syndrome in two siblings

Abstract

The Scimitar syndrome is a complex association of cardiovascular and bronchopulmonary abnormalities, with the main feature a partial or total anomalous right pulmonary venous drainage to the inferior vena cava. A number of cases that lack of all the features of the typical syndrome have been described as Scimitar variant, but the incidence is rare. Familial occurrence is exceptional and limited to few cases in literature. We report two sibling diagnosed with an uncommon variant of the Scimitar syndrome.

Keywords: Anomalous pulmonary venous drainage; Scimitar; Scimitar syndrome; Scimitar variant; familial Scimitar syndrome.

Figure 1

Patient 1, anterior projection of selective angiogram into the aorto-pulmonary collateral during coil embolization (C). There is an anomalous right Scimitar vein (SV) draining to the inferior vena cava (IVC) which was stenosed at the junction with inferior vena cava and connected to the right upper pulmonary vein (RUPV) draining to left atrium by a large and tortuous fistula (*). There is dextroposition of the heart

Figure 2

Patient 2, selective injection in IVC showing anomalous drainage of all the right pulmonary veins (RPV) to the hepatic vein with severe pulmonary vein stenosis and a collateral vessel between the right pulmonary vein and artery (*)

Figure 3

Selective injection into the descending aorta showing a moderate size aorto-pulmonary collateral (APC) arising from Coeliac axis (CA) and supplying the lower part of right lower lobe. SMA = superior mesenteric artery (b) (bis): Selective injection into the descending aorta showing a moderate size aorto-pulmonary collateral (APC) supplying the lower part of the right lower lobe (RLL)