Moderate-term effect of epoprostenol on severe portopulmonary hypertension.

We examined the effect of continuous intravenous infusion of epoprostenol (35 ng/kg/min) on severe portopulmonary hypertension caused by biliary atresia. Pulmonary hemodynamics improved and brain natriuretic peptide and human atrial natriureic peptide decreased to normal values during epoprostenol therapy. However, the improvement in pulmonary hemodynamics was not sufficient to permit liver transplantation. Our patient was obliged to stop epoprostenol therapy because of financial problems and epoprostenol was tapered off safely over 6 weeks.