Abstract

Congenital extra hepatic port systemic shunt, is a rare clinical entity which can lead to hepatopulmonary syndrome wherein blood from portal vein directly drains into systemic circulation, causing an alteration in metabolism of pulmonary vasoactive substances, pulmonary vasodilatation, diffusion-perfusion defects, and eventually, arterial hypoxemia due to either an increase in pulmonary vascular resistance or the creation of arteriovenous malformations.

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