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Progressive Portopulmonary Hypertension After Liver Transplantation Treated With Epoprostenol*

Albert Lim Rafanan, MD; Janet Maurer, MD, FCCP; Atul C. Mehta, MD, FCCP; Robert Schilz, DO, PhD, FCCP
Chest. 2000;118(5):1497-1500. doi:10.1378/chest.118.5.1497
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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.


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