What is the efficacy of epoprostenol for the treatment of pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Epoprostenol is a synthetic prostacyclin that is available only as an intravenous infusion. It induces relaxation of vascular smooth muscle and inhibits its growth and platelet aggregation through the increase in intracellular cyclic adenosine monophosphate (cAMP). [36, 37, 38] A prospective, randomized, open-label trial was conducted on 81 patients with PAH. After 12 weeks, epoprostenol therapy led to functional improvement, as shown by an improved 6-minute walk test and a decrease of 8% in mean pulmonary arterial pressure. [36] Intravenous epoprostenol improved exercise tolerance, hemodynamics, and long-term survival in another cohort of 178 patients with PAH, as compared with historical controls. [38] Another trial, in which a cohort of 162 patients was studied after 1 year of receiving epoprostenol therapy, confirmed that patients’ clinical function improved significantly, even though improvements in hemodynamic measures were modest. Improvement with epoprostenol has also been reported in patients who had PAH associated with congenital left-to-right cardiac shunts, portal hypertension, and HIV infection. [39] Of note, epoprostenol is also the only treatment that has shown a mortality reduction, although this was in a single randomized controlled trial. [36] Epoprostenol is administered only by continuous intravenous infusion via a portable infusion pump connected to a permanent catheter. Compared with other prostacyclin-based therapies, epoprostenol has a very short half-life (3-5 min) and is not stable at room temperature for more than 8 hours. Sudden drug interruption can thus be life-threatening. The dosage is determined during a dose-effect study performed in a catheterization laboratory or ICU. The selected dosage produces maximum vasodilation with minimum systemic hypotension. Common adverse effects of epoprostenol include jaw pain, headache, diarrhea, flushing, leg pain, and nausea, although these are generally mild and dose-related. Other complications include catheter-related sepsis, pump failure, or dislocation of the central venous catheter.


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