PMC4322404_01
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
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F1
At 6 months the patient underwent cardiac catheterization and bronchoscopy under general anesthesia and this revealed a Scimitar variant consisting of: Severe stenosis of the right pulmonary vein at its connection with the inferior vena cava, atrio-ventricular and ventriculo-arterial concordance, no interatrial communication, with a fistulous communication of the anomalous vein to the right upper pulmonary vein which drained normally to the left atrium [Figure 1].
PMC4322404_02
PMC4322404_03
PMC4322404_04
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 (*)
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F2
Postnatal assessment, including cardiac catheterization, confirmed the diagnosis of Scimitar variant with total anomalous right pulmonary venous drainage to the inferior vena cava below diaphragm, although there was severe obstruction of the anomalous vein at the junction with inferior vena cava; a collateral vessel was seen between the right pulmonary vein and artery through selective injection into the inferior vena cava [Figure 2].
PMC4322404_05
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)
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F3
There was also right lung hypoplasia with the medium and lower lobe supplied by two aorto-pulmonary collaterals arising from the descending aorta, above the diaphragm and from coeliac axis [Figure 3].