The status of pulmonary arterial hypertension in 2008.

Pulmonary Arterial Hypertension (PAH) is a devastating disease that, until recently, had no effective medical therapy. In 1996, a prostacyclin, epoprostenol, was approved; since then, several therapies, including prostacyclin analogues and agents that modulate other vasoactive pathways – endothelin receptor antagonists and phosphodiesterase inhibitors – have received approval. In this issue of Circulation, Galiè et al report the results of the 2 pivotal trials of the endothelin receptor antagonist, ambrisentan, that lead the US Food and Drug Administration to approve this agent in June 2007.1