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

Sweta Mohanty, Shreesha Maiya, Dheeraj Kumar Tiwari
Narayana Health.
India

Cardiology in the Young
Cardiol Young 2025;
DOI: 10.1017/S1047951125109220

Abstract
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.

Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease

Age Focus: Pediatric Pulmonary Vascular Disease

Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No

Scroll to Top