Congenital extrahepatic portosystemic shunt with partial anomalous pulmonary venous return and aneurysmal dilation of the pulmonary artery: successful management of a complex multisystem congenital anomaly.

Congenital extrahepatic portosystemic shunt (CEPS) is an uncommon condition in which the portal vascular system drains into the systemic veins. We describe an unusual case of CEPS with congenital heart disease, presenting with aneurysmal enlargement of the pulmonary artery and symptoms of airway compression, highlighting the importance of evaluating for additional pathology in case of unexplained oxygenation defect or pulmonary hypertension.