Pulmonary arterial hypertension (PAH) is characterized by a progressive increase in pulmonary vascular resistance due to vascular remodeling, vasoconstriction and thrombus formation in the distal pulmonary arteries (PAs).1–3 Currently, 5 classes of drugs, prostacyclin analogues, prostacyclin receptor agonists, endothelin receptor antagonists, phosphodiesterase-5 inhibitors and soluble guanylate cyclase stimulators, are clinically used for the treatment of PAH. This evidence concerns the gene PTGIR and pulmonary arterial hypertension.