Prostacyclin Therapy for Pulmonary Arterial Hypertension

Abstract

The first class of agents approved specifically for the therapy of pulmonary hypertension in the United States, the prostacyclins have assumed a key treatment role, especially via the intravenous route for class IV patients. The best established agent for efficacy and the only one shown to significantly improve survival is epoprostenol via continuous intravenous infusion. Epoprostenol also improves symptoms, exercise capacity, and pulmonary hemodynamics in IPAH patients. While the literature is not as voluminous in patients with PAH associated with other disorders, epoprostenol therapy improves the symptoms of PAH in many of these patients. Enthusiasm for epoprostenol must be tempered by its cumbersome intravenous delivery system and inherent risks. Newer prostacyclins include treprostinil, available in subcutaneous and intravenous forms, and inhaled iloprost. Each has established efficacy in randomized trials. Subcutaneous treprostinil has been plagued by infusion site pain but is a good alternative for those who can tolerate it. Inhaled iloprost is being used increasingly because the inhaled route has fewer systemic side effects and improves oxygenation compared to the intravenous route, and an improved administration system makes it more tolerable for patients. Whichever of the prostacyclins is chosen, patients are monitored closely thereafter because of the many potential complications and since most forms require timely dose increases because of tolerance.

Part of CIRS Nexus · Curated by Lauren Lowenstein, The Biotoxin Lady