Living related liver transplantation for biliary atresia with portopulmonary hypertension: case report.

Portopulmonary hypertension is a complication of end-stage liver disease that adversely affects the outcome of liver transplantation (LT). We report a case of living related LT who developed severe pulmonary hypertension during and after LT. This 16-year-old girl suffered from biliary atresia, having undergone a portoenterostomy at 60 days of age, at the time of discovery of liver cirrhosis. She had been admitted to a local hospital several times for episodes of esophageal variceal bleeding. Neither dyspnea nor cyanosis was discerned until LT. Although pulmonary hypertension (PH) was disclosed by echocardiogram upon preoperative evaluation, we did not consider this a contraindication for LT, because the PH was mild. She underwent living LT from her father (graft volume/recipient body weight ratio: 0.99%). After induction of anesthesia for LT, a pulmonary flotation catheterization showed severe PH (>40 mm Hg). The pulmonary artery pressure continued to be elevated during surgery, although it was possible that her severe scoliosis affected the data. Hyperbilirubinemia was observed after LT, despite good liver function tests. On postoperative day 12, a portal vein thrombosis was detected requiring emergency thrombectomy and splenectomy. Her general condition worsened after the second surgery. She died due to cardiopulmonary failure. Autopsy showed marked hypertrophy of the right ventricle with intimal thickening in the pulmonary artery. In this case, the underestimated PH might have resulted in the unfortunate outcome. Before LT, PH should be carefully evaluated by measures including invasive assessment.