Liver transplantation in patients with severe portopulmonary hypertension treated with preoperative chronic intravenous epoprostenol

Portopulmonary hypertension (PPHTN) is no longer an absolute contraindicati on to orthotopic liver transplantation (OLT). The pre-OLT management of pat ients with PPHTN requires early diagnosis and chronic therapy with intraven ous epoprostenol to decrease pulmonary vascular resistance (PVR). Close fol low-up is necessary to reassess pulmonary artery pressures (PAPs) and evalu ate right ventricular (RV) function. This assists in the optimal timing of OLT. Successful management also necessitates reassessment of pulmonary arte ry hemodynamics just before OLT, with clearly defined parameters used to de termine whether to proceed. Even with the intraoperative and postoperative availability of potent pulmonary vasodilators, clinical management may be s uboptimal in reducing PAP. Adequate reduction in PVR and improvement in RV function in response to chronic epoprostenol therapy may facilitate success ful OLT. We present a case report and review the limited experience with th is treatment.