Advances in pharmacotherapy over the past three decades have led to the approval of sixteen unique medications or medication formulations for patients with PAH, including phosphodiesterase-type 5 inhibitors (PDE5i), soluble guanylate cyclase (sGC) stimulators, endothelin receptor antagonists (ERAs), prostacyclin analogues (PCAs), and selective prostacyclin receptor (IP) agonists [2,3]. The gene discussed is PTGIR; the disease is pulmonary arterial hypertension.