Infra-cardiac TAPVC with an intrahepatic portosystemic shunt: A rare association

Abstract

Total anomalous pulmonary venous connection (TAPVC) is an uncommonly encountered cyanotic congenital heart disease characterized by anomalous drainage of the pulmonary veins. Concomitant venous anomalies are rarely found in these patients. We present a case of an infra-cardiac TAPVC with an intrahepatic portosystemic shunt, where a vascular channel was seen between the middle hepatic vein and the left branch of the portal vein with fusiform dilatation of the latter, diagnosed on computed tomography angiography.

Keywords: Imaging; infra-cardiac; intrahepatic shunt; portosystemic shunt; total anomalous pulmonary venous connection.

Figure 1

Computed tomography angiography images (a-d). Volume rendered image (a) showing the common vertical channel (white arrow) draining into the main portal vein; dilated left branch of the portal vein (yellow arrow) and vascular shunt (white asterisk). Sagittal (b) and axial (c) images showing the vascular shunt (asterisk) between the middle hepatic vein (red arrow) and dilated left branch of the portal vein (yellow arrow). Axial image (d) showing fusiform dilatation of the left branch of the portal vein (yellow arrow). Common vertical channel is also seen in the axial images (white arrows in c and d)