Rare cause of neonatal pulmonary hypertension: Congenital intrahepatic portosystemic shunt through an aneurysm

Congenital portosystemic shunt is a rare congenital anomaly with abnormal communication between portal venous and systemic venous systems. It can be intrahepatic or extrahepatic. Typically, the intrahepatic shunts are managed conservatively as many of them close spontaneously. We present and discuss clinical, radiological findings of an intrahepatic shunt showing the early occurrence of pulmonary arterial hypertension in the neonatal period which required therapeutic intervention.