The Future of PAH Treatment

Pulmonary arterial hypertension (PAH) is a devastating disease mediated by vasoconstriction and vascular remodeling of the pulmonary vasculature. Current therapies target the imbalance of vasoconstrictors and vasorelaxants in 3 pathways: nitric oxide, prostacyclin, and endothelin. While these have extended lifespans for PAH patients, significant morbidity and mortality remains. Notably, the progress in PAH therapy for over a decade has utilized these same 3 pathways. Fortunately, several new treatment options utilizing different mechanisms are emerging and will be reviewed here.