Current therapeutics for pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is characterized by a progressive rise in pulmonary vascular resistance resulting from vascular remodeling, vasoconstriction, and cellular proliferation. For patients diagnosed with PAH, the prognosis is poor, with an approximate 9-15% annual mortality rate.The goal of medical therapy is to improve survival and patients' quality of life. Although PAH is an incurable orphan disease with a high mortality rate, current treatment strategies have led to considerable gains, including exercise capacity, hemodynamic and time to clinical worsening. General disease management options include avoidance of salt in the diet, oxygen, appropriate vaccinations and routine health maintenance, and avoidance of pregnancy. Traditional treatments include warfarin, diuretics, and calcium channel blockers (if patients are deemed "responders" during cardiac catheterization). Currently, three classes of drugs are approved for the treatment of PAH based on results from clinical trials prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors. Clinical practice patterns have shifted in favor of earlier diagnosis, aggressive front line treatment, and upfront versus stepwise combination therapy. Future drug development targeting other molecular pathways of pulmonary vascular disease is essential for advancing our understanding of this disease. The purpose of this review is to summarize recent guidelines for the management of pulmonary arterial hypertension, and highlight clinical topics for the primary care physician.