P0377 HEPATOPULMONARY SYNDROME IN CHILDREN: A SINGLE CENTRE EXPERIENCE

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Introduction: Hepatopulmonary syndrome (HPS) is a rare complication of chronic liver disease or portal hypertension. The clinical presentation is dyspnoea on exertion or overt cyanosis. In the past, this complication contraindicated liver transplantation (LT). However, good outcome with reversal of symptoms after LT has been described. Methods: Single centre experience of management and outcome of HPS in 12 children. Results: Twelve children were diagnosed to have HPS on macroaggregated albumin (MAA) lung scintigraphy over a 15 year period (1988–2003), underlying liver disease being biliary atresia (BA) in 6, progressive intrahepatic cholestasis in 2, cryptogenic cirrhosis, billiary cirrhosis, portal vein sclerosis and alpha 1 antitrypsin deficiency in 1 each. Biliary atresia splenic malformation (BASM) syndrome was present in 3. There were 8 girls (65%) with a median (range) age at diagnosis of 6.6 years (1.4–15.4 years). All patients had clinical evidence of portal hypertension with 4 having a history of gastrointestinal bleeding. The presenting symptoms were dyspnoea (7), cyanosis (9)and clubbing (10). All children with BA had cleared their jaundice. Median (range) oxygen saturation by pulse oxymetry was 90% (56–95%) with mild increase on supplemental facial oxygen. MAA scintigraphy revealed median pulmonary shunting of 22% (range 4–49%). The degree of shunting was mild (<20%) in 5, moderate (20–40%) in 4 and severe (>40%) in 3. Cardiac echography revealed normal function of the heart in all. All children were listed for LT. Six children required oxygen therapy prior to LT. One child with BASM and a moderate shunt died of sepsis while awaiting LT. One child is currently listed for LT. Ten children underwent LT 6–20 months (median 12 months) after diagnosis of HPS. Six children were extubated after 24 hours with the longest period of ventilation being 10 days. Median (range) oxygen requirement after LT was 21 days (1–45 days). Two children died 1 week and 3.2 years post LT of multi-organ failure and post transplant lymphoproliferative disorder respectively. Nine children are alive and well 5.25 years (range 1 month-12.8 years) after diagnosis of HPS. Conclusion: HPS is reversible after LT and does not appear to complicate the postoperative course. Female preponderance in our study group remains unexplained but may be an effect of female sex hormones on pulmonary circulation.