Pharmacotherapy for pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) continues to be a condition associated with a high morbidity and mortality despite advances in treatment strategies over the past 25 years. The 6th World Symposium on Pulmonary Hypertension defines pulmonary hypertension as a mean pulmonary arterial pressure >20 mmHg (1). Currently approved therapies for PAH target three primary pathways that are key in the pathogenesis of the disease: the nitric oxide, prostacyclin and endothelin pathways (see Figure 1). The imbalance of vasoactive mediators plays a primary role in the development and progression in the proliferative pathological changes in PAH. PAH-specific therapies have been available since the mid-1990s. Further refinement in appropriate utilization of these medications as well as combination therapy has been critical in the last decade. In this articles, current therapies for the PAH are discussed as well as defining patients appropriate for therapy