Long-term outcome and management of hepatopulmonary syndrome in children

We aim to report a single center experience of the management and long term outcome of HPS in pediatric liver transplant recipients. A retrospective review of children with HPS from 1990 to 2004. Inclusion criteria: liver disease or portal hypertension, hypoxemia (PaO2 <70 mmHg or SaO(2) <95%) and intrapulmonary shunting documented by macroaggregated albumin scan ratio of > 4% (classified mild group [<20%], moderate group [20-40%] and severe group [> 40%]). Resolution of HPS post-liver transplant was defined as PaO2 > 70 mmHg or SaO(2) > 95%. Eighteen children (six male [34%], median age at diagnosis of HPS 8.6 [1-15.5] yr) had HPS: biliary atresia (n = 8), idiopathic biliary cirrhosis (n = 4), progressive intrahepatic cholestasis (n = 2), miscellaneous (n = 4). The majority had mild shunting (n = 8). Fourteen underwent transplantation with resolution of HPS in 13. Six developed complications: hepatic artery thrombosis (n = 4), biliary (n = 2). Four children died (28%), two pretransplant. There was a tendency towards shunt fraction worsening to a slower degree over time. One-yr survival rate post-transplant was 93%. Median PaO2 was significantly lower in non-survivors compared to survivors (43 vs. 55.2 mmHg, p = 0.03). There was correlation between oxygen parameters pretransplant and time to HPS resolution post-transplant. HPS is reversible after transplant, but is associated with increasing mortality and morbidity.