It should be noted that PAH therapy in Russia is limited to endothelin receptor antagonists (ERA) (bosentan, ambrisentan, macitentan), phosphodiesterase type 5 inhibitor (PDEi-5- sildenafil), prostanoids (inhaled iloprost), IP-receptors agonist (selexipag), and soluble guanylate cyclase stimulator (sGC—riociquat). The gene discussed is SGCB; the disease is pulmonary arterial hypertension.