Contemporary pharmacotherapies involving nitric oxide, prostacyclin, and endothelin receptor signaling pathways

The progressive disorder of pulmonary arterial hypertension (PAH) is marked by a complex pathobiology of pulmonary vascular remodeling, including dysregulated cellular proliferation and inappropriate local and systemic inflammatory responses. The various approved therapies for PAH currently target some of these pathways and have collectively led to improvements in symptoms and overall survival. Specifically, each approved therapy targets one of either the prostacyclin, endothelin, or nitric oxide pathways, respectively. Moreover, each of these broad classes of drugs and the individual drugs within each class has been studied in a variety of contexts and each has unique characteristics relevant to the clinician caring for the PAH patient. In this chapter we provide an in depth review of the currently available pharmacotherapies targeting the prostacyclin, endothelin, and nitric oxide signaling pathways.