Current and future therapeutic targets for pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) remains a poorly understood disorder with dire consequences. Progression of disease often leads to right heart failure and death without lung transplantation. Limited therapeutic strategies exist for the treatment of PAH and current medical treatment consists of two major categories, supportive care therapeutics and pulmonary vasculature targeted therapy. Supportive care includes anticoagulants and inotropic agents, while pulmonary vasculature targets currently available include endothelin antagonists, phosphodiesterase inhibitors and prostacyclin analogs. While these therapies have shown efficacy in hemodynamic and functional outcomes; halting the progression of disease, reversal, and clinical cure has been elusive. Combination therapy and newer agents may provide innovative insight into the disease process and ultimately change the prognosis of this fatal disease. By utilizing specific targets on the cell membrane, second messengers systems or signaling peptides, novel therapeutic strategies will hopefully emerge. In this review we discuss the currently available therapeutic options and their pulmonary vasculature targets as well as some future therapeutic targets that have promising results in human trials.