The evolving treatment landscape of pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a severe disease with poor prognosis and shortened life expectancy. Treatment has traditionally involved the sequential use of endothelin receptor agonists, prostacyclin therapies, and nitric oxide pathway modulators, which each have distinct mechanisms of action leading to pulmonary vasodilation, and improvement in exercise capacity, hemodynamic measures, and clinical outcomes for patients with PAH. This article provides a review of goals of therapy in PAH, determinants of prognosis and levels of patient risk, and additional factors that guide treatment decision making. Recent research in combination therapies has created a paradigm shift in the treatment of PAH and will be reviewed. Additionally, recent updates to the American College of Chest Physicians guidelines will be reviewed along with the updated evidence-based treatment algorithm. Finally, trial data will be evaluated for the recently developed agent selexipag and improved treprostinil delivery formulations that may provide enhanced convenience.