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.