Progressive portopulmonary hypertension after liver transplantation treated with epoprostenol

Abstract

Portopulmonary hypertension (PPHTN) is an uncommon complication of advanced liver disease. Epoprostenol has been effective in the treatment of PPHTN and has been used as a bridge to orthotopic liver transplantation (OLT). The role of OLT in the reversal of PPHTN is unclear. We report a case of severe PPHTN (mean pulmonary artery pressure of 45 mm Hg) that progressed after OLT. Acute dosing with epoprostenol improved the pulmonary vascular resistance by 55% and the cardiac index by 134%. Hemodynamic and symptomatic improvements were maintained after 18 months of long-term treatment with epoprostenol. This is the first reported case of a successful favorable outcome after treatment for progressive PPHTN after OLT. Our case report complements previous reports by highlighting the potential effective use of epoprostenol as a definitive treatment for PPHTN.