Case report on the successful use of a new selective prostacyclin IP receptor agonist, selexipag, in a patient with idiopathic pulmonary hypertension

Idiopathic pulmonary arterial hypertension (IPAH) is a rather rare cardiovascular disease of unknown origin and, at the same time, the most common form of pulmonary arterial hypertension (PAH). It is characterized by increased mean pulmonary artery pressure of ≥ 25 mm Hg and increased pulmonary vascular resistance of > 3 Wood units. One of the key components of IPH pathogenesis is the disorder in the prostacyclin pathway leading to hypertrophy of the smooth muscle and endothelial cells, fibrotic changes, inflammatory response and vasoconstriction, which results in small artery and arteriole remodeling. For severe cases of PAH, in Russia only used one drug acting on the prostacyclin pathway, i.e., iloprost for inhalation. However, this therapy has a number of restrictions on the use. Selexipag is the first selective oral prostacyclin IP-receptor agonist intended for the treatment of patients with PAH. The efficacy and safety of Selexipag in patients with PAH were investigated in GRIPHON study, the largest-scale clinical trial ever conducted in patients with pulmonary hypertension. The publication presents a case of successful use of Selexipag as a part of the combination therapy in a patient with IPAH.