Key result
Targeted therapies for pulmonary arterial hypertension acting on the nitric oxide, endothelin, and prostacyclin pathways improve exercise capacity and hemodynamics, though disease progression remains significant.
This review summarizes the current therapeutic agents available for the medical management of pulmonary arterial hypertension, highlighting pathways such as prostacyclin, endothelin, and nitric oxide.
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May support targeted PAH therapies for functional and hemodynamic gains; leaves open need for progression-halting agents.
Pulmonary arterial hypertension (PAH) is an uncommon, progressive and life threatening disease characterized by a proliferative vasculopathy of the small muscular pulmonary arterioles resulting in elevated pulmonary vascular resistance and eventually right ventricular failure. An increasing understanding of the pathobiology of PAH and its natural history has led to the development of numerous targeted therapies. Despite these advances there is significant progression of disease and the survival rate remains low. This article reviews the agents currently available for the medical management of PAH.
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Hahn et al. (2016) conducted a review in Pulmonary arterial hypertension. Targeted therapies for PAH (prostanoids, endothelin receptor antagonists, PDE5 inhibitors, sGC stimulators) was evaluated. Targeted therapies for pulmonary arterial hypertension acting on the nitric oxide, endothelin, and prostacyclin pathways improve exercise capacity and hemodynamics, though disease progression remains significant.
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