Pharmacologic therapy for pulmonary artery hypertension

Purpose of review Pulmonary arterial hypertension (PAH) is a disease that carries a significant mortality left untreated. This article aims to review pharmacotherapeutics for PAH. Recent findings PAH-specific therapies have evolved over the last three decades and have expanded from one therapy in the 1990s to 14 FDA-approved medications. Current therapies are directed at restoring the imbalance of vasoactive mediators that include nitric oxide, endothelin and prostacyclin. Although these agents are effective as monotherapy, recent trials have promulgated the strategy of upfront combination therapy. The availability of oral prostacyclin agonists has also allowed for expanded treatment options. Risk assessment is vital in guiding therapy for PAH patients. There is ongoing focus on targeting pathological mechanisms of the disease via novel therapies and repurposing existing drugs. There is an array of medications available for the treatment of PAH. Prudent combination of therapies to maximize treatment effect can improve morbidity and mortality. This article reviews the data supporting these therapies and attempts to outline an approach to patient management.