Pharmacotherapy of refractory Pulmonary arterial hypertension.

By utilizing serial risk assessment to identify patients remaining intermediate or high-risk, more patients are likely to survive longer. This is done by earlier use of combination or triple therapy with prostacyclin drugs. Current medications for PAH are all essentially vasodilators that improve physiology, but do not truly modify the disease process. The potential application of new investigational medications is exciting as they work by novel pathways likely to change the landscape of refractory PAH treatment.