ABSTRACT
Prostacyclin therapy was the first therapy approved specifically for pulmonary arterial hypertension (PAH), and it has transformed the care of patients with this devastating disease. Over 30 years ago epoprostenol (PGI2), a stable freeze-dried preparation of prostacyclin, was shown to reduce symptoms and improve hemodynamics in a woman with severe primary pulmonary hypertension (PPH), now referred to as idiopathic pulmonary arterial hypertension (IPAH), awaiting lung transplant. Following relatively small clinical trials and a pivotal trial that established the safety and efficacy in IPAH, intravenous epoprostenol (Flolan) was approved for clinical use by the U.S. Food and Drug Administration (FDA) in 1995 (1). The effectiveness of prostacyclin, a vasodilator and inhibitor of platelet aggregation, reflects the mechanisms believed to contribute substantially to the development of PAH. The evolution of prostanoid therapies with FDA approval has been rapid and now includes intravenous epoprostenol (Flolan,
Veletri, and generic), intravenous and subcutaneous treprostinil (Remodulin), inhaled iloprost (Ventavis), and inhaled treprostinil (Tyvaso) and oral treprostinil (Orenitram). The prostacyclin receptor agonist, selexipag, has also been studied in phase 3 pivotal trials and has been submitted for regulatory approval.
