Transcatheter closure of congenital extrahepatic portosystemic shunt to right atrium causing severe pulmonary hypertension in a child: A case report

Abstract

Congenital extrahepatic portosystemic shunt is an abnormal connection between portal vein (prior to its branching) and one of the systemic veins. It is a rare cause of pulmonary hypertension. We report a 2-year-old child who presented with severe pulmonary artery hypertension and a unique form of congenital extrahepatic portosystemic shunt draining directly into right atrium. The shunt was successfully closed percutaneously, and pulmonary artery pressure fell from severe pulmonary artery hypertension to moderate pulmonary artery hypertension on follow up.