HEPATOPULMONARY SYNDROME IN CHILDREN WITH BILIARY ATRESIA- PROGRESSION, TRANSPLANTATION, AND OUTCOME.

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706 In hepatopulmonary syndrome (HPS), pulmonary arterial vasodilatation and arterio-venous shunting produce hypoxemia in patients with cirrhosis. We describe 4 children with biliary atresia and subsequent development of HPS. Outcome of liver transplantation in two of these patients is described. Our Pediatric GI Registry contains the names of 120 children with biliary atresia. Of these, 4 children who had a successful Kasai operation later presented with dyspnea, fatigue and digital clubbing which led to the diagnosis of HPS. All 4 had slowly progressive cirrhosis and portal hypertension. The diagnosis of HPS was confirmed by bubble contrast echocardiography in 3 and scintigraphy in 1. Dyspnea and exercise intolerance became disabling over a 1-2 year period after diagnosis in all of these children. (Table)TableSummary: Hepatopulmonary syndrome developed in 4/120 (3%) patients with biliary atresia. HPS was the major morbidity of their cirrhosis and the indication for OLT. Hypoxemia and dyspnea/exercise limitation usually progressed over a 1-2 year period after diagnosis. In the two children who received a liver transplant for HPS, symptoms of hypoxemia resolved over 2 months and oxygen saturations on room air normalized. Conclusions: HPS is a more common complication of cirrhosis due to biliary atresia than previously recognized. Liver transplantation can reverse HPS. Children with cirrhosis secondary to biliary atresia should be monitored closely for signs and symptoms of this complication since progression can be expected in 1-2 years after diagnosis.