title: 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.
authors.affiliation: Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, OH 44195, USA.
authors.forename: A L
authors.initials: AL
authors.lastname: Rafanan
authors.forename: J
authors.initials: J
authors.lastname: Maurer
authors.forename: A C
authors.initials: AC
authors.lastname: Mehta
authors.forename: R
authors.initials: R
authors.lastname: Schilz
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D001794:Blood Pressure / Q000187:drug effects; D002302:Cardiac Output / Q000187:drug effects; D018450:Disease Progression; D011464:Epoprostenol / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006521:Hepatitis, Chronic / Q000601:surgery; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy* / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D016031:Liver Transplantation; D011651:Pulmonary Artery; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000187:drug effects
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol