Successful liver transplantation in a child with biliary atresia and hepatopulmonary syndrome.

Hepatopulmonary syndrome (HPS) is characterized by hypoxemia in patients with severe chronic liver disease and pulmonary vasodilatation in the absence of primary cardiac or pulmonary disease. Severe hypoxemia resulting from HPS is generally considered a contraindication to liver transplantation. We describe the case of a 6-year-old girl with biliary atresia complicated with HPS who was successfully treated with liver transplantation. Cyanosis and dyspnea had initially developed at the age of 5 years. Arterial blood gas showed a PaO2 of 46.6 mm Hg on room air. The diagnosis of HPS was confirmed by contrast echocardiography, lung perfusion scan with 99mTc macroaggregate albumin, and pulmonary angiography. The lung scan revealed an intrapulmonary shunt of 24%. She underwent living donor liver transplantation and received a left lateral segment graft from her mother. One year after successful liver transplantation, she had normal arterial oxygen saturation and a normal lung scan without intrapulmonary shunting. This case demonstrates that HPS associated with end-stage liver disease is potentially curable by liver transplantation.