SUCCESSFUL USE OF CHRONIC EPOPROSTENOL AS A BRIDGE TO LIVER-TRANSPLANTATION IN SEVERE PORTOPULMONARY HYPERTENSION

Background. Portopulmonary hypertension, defined as mean pulmonary art ery pressure >25 mmHg in the presence of a normal pulmonary capillary wedge pressure and portal hypertension, is a known complication of end -stage liver disease that has been associated with high morbidity and mortality at the time of liver transplantation. We have recently repor ted the successful treatment of portopulmonary hypertension with chron ic intravenous epoprostenol and now report the first patient with seve re portopulmonary hypertension successfully treated with epoprostenol who subsequently underwent successful liver transplantation. Methods. A patient with severe portopulmonary hypertension was treated with int ravenous epoprostenol, 23 ng/kg/min, for a 4-month period, after which the portopulmonary hypertension resolved and the patient underwent su ccessful liver transplantation. Results. The patient was discharged, c ontinues to do well, and at 3 months is off epoprostenol with near nor mal pulmonary artery pressures. Conclusions, Chronic epoprostenol, in conjunction with a multidisciplinary, well-planned perioperative evalu ation and treatment plan, may be the answer to a heretofore untreatabl e disease.