Liver transplantation for patients with severe portopulmonary hypertension (PPHTN) has been associated with high mortality. We conducted perioperative management of two patients with severe PPHTN for living-donor liver transplantation. The first case was a 17-year-old male with biliary atresia. He developed dyspnea at the age of 14, for which he was treated with intravenous epoprostenol for 8 months. As a result, the mean pulmonary artery pressure (MPAP) was reduced from 61 to 40 mmHg. Intraoperatively, he was treated with intravenous epoprostenol and nitric oxide (NO) inhalation. His intraoperative course was uneventful but he died from right heart failure on postoperative day (POD) 11. The second case was a 6-year-old girl with biliary atresia. When she was 5 years old, examination for a persistent cough revealed MPAP of 49 mmHg. Neither intravenous epoprostenol nor NO inhalation was effective, and she twice showed transient pulmonary hypertension during the operation. She was extubated 14 hours after the surgery, transferred out of ICU on POD 3 and discharged from the hospital on POD 99. When we compare the two cases, the factors responsible for the success of the management of the second case appear to be early extubation and the short duration of PPHTN.
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