Progressive portopulmonary hypertension after liver transplantation treated with epoprostenol

Chest. 2000 Nov;118(5):1497-500. doi: 10.1378/chest.118.5.1497.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antihypertensive Agents / therapeutic use*
  • Blood Pressure / drug effects
  • Cardiac Output / drug effects
  • Disease Progression
  • Epoprostenol / therapeutic use*
  • Female
  • Follow-Up Studies
  • Hepatitis, Chronic / surgery
  • Humans
  • Hypertension, Portal / drug therapy*
  • Hypertension, Portal / physiopathology
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / physiopathology
  • Liver Transplantation*
  • Pulmonary Artery
  • Treatment Outcome
  • Vascular Resistance / drug effects


  • Antihypertensive Agents
  • Epoprostenol