Treatment of Patients Undergoing Liver Transplant for Portopulmonary Hypertension with Oral Therapy

Introduction: In patients undergoing liver transplantation for portopulmonary hypertension, IV epoprostenol was the first line drug and standard of care prior to oral medications; however, outcomes between these 2 approaches have not been compared. We analyzed the efficacy of oral medication in the treatment of portopulmonary hypertension and compared it with prior IV medications. Methods: We examined patients with portopulmonary hypertension receiving oral treatment followed by liver transplantation (2007-2019). We analyzed the efficacy of oral treatment on cardiopulmonary hemodynamic and posttransplant outcome. Duration of treatment pre and posttransplant and outcomes were also recorded. These parameters were then compared with our previously recorded data of patients treated with IV treatment followed by liver transplant between 1997 to 2005. Results: Oral therapy: 13 patients (median age of 58 years (interquartile range, IQR 52-60), 54% male) received oral treatment. The 3 classes of agents used were phosphodiesterase 5 inhibitors (N=6) endothelin receptor antagonist (N=5), and prostacyclin receptor agonist (N=4). All except 3 needed more than 1 agent. IV therapy: 11 patients (median age 52 years (IQR 46-56), 73% male) received IV Epoprostenol. Comparison: Patients on oral treatment underwent transplant after the mean duration of 15.8 months as compared to 9.6 months of IV treatment before transplant. The median time to normalization was 3 months (1-11 mo.) The mean duration of treatment was 55.5 months post liver transplant in oral treatment group and all except 1 remained on oral treatment until the last follow-up. The mean duration of IV epoprostenol was 7.1 months post liver transplant. Nine patients were taken off IV treatment and all remained asymptomatic except 1 who was later started on oral treatment. Survival: 1-, 2- and 5-years post-transplant survival of 100% on oral treatment compared to 90.9, 80.8% and 67.3% on IV treatment group (Figure 1). Conclusion: Both IV epoprostenol and oral medications are equally efficacious in improving pulmonary pressure prior to liver transplant though improvement was much faster in the IV group as compared to oral group. Posttransplant, most of the patients were taken off IV treatment as compared to oral treatment. Survival was better in oral group as compared to IV group, though may be related to era effect. Subsequent analyses are needed comparing cost and advances in posttransplant management over time.Figure 1.: Survival.