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

Hepatopulmonary syndrome (HPS) is characterized by hypoxemia in patients wi th severe chronic liver disease and pulmonary vasodilatation in the absence of primary cardiac or pulmonary disease. Severe hypoxemia resulting from H PS is generally considered a contraindication to liver transplantation. We describe the case of a 6-year-old girl with biliary atresia complicated wit h 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 Tc-99m macroaggrega te albumin, and pulmonary angiography. The lung scan revealed an intrapulmo nary shunt of 24%. She underwent living donor liver transplantation and rec eived a left lateral segment graft from her mother. One year after successf ul liver transplantation, she had normal arterial oxygen saturation and a n ormal lung scan without intrapulmonary shunting. This case demonstrates tha t HPS associated with end-stage liver disease is potentially curable by liv er transplantation.