Porto pulmonary hypertension due to progression of secondary biliary cirrhosis following Kasai procedure

Porto pulmonary hypertension is the presence of pulmonary arterial hypertension in patients with advanced liver disease and portal hypertension. It affects minority of advanced liver disease patients without a significant correlation between hepatic impairment and severe porto pulmonary hypertension. It is a rare complication of portal hypertension following successful portoenterostomy. We present a case of a seventeen-year-old male with a past history of Kasai procedure for congenital biliary atresia, presenting with severe pulmonary hypertension as a complication of cirrhosis. He was defaulted during childhood follow up and was asymptomatic during last 17 years which has led to silent progression of secondary biliary cirrhosis. Hence this highlights the importance of long-termfollow up after Kasai procedure.