TABLE 3

Approved pulmonary arterial hypertension therapies

Therapeutic targetAgents approved for clinical use in pulmonary arterial hypertension
Prostacyclin pathwayProstanoids
 Epoprostenol (intravenous)
 Treprostinil (subcutaneous and oral)
 Iloprost (inhaled)
Selective IP prostacyclin receptor agonist
 Selexipag (oral)
Endothelin-1 pathwayDual endothelin receptor antagonists
 Bosentan (oral)
 Macitentan (oral)
Selective endothelin type A receptor antagonists
 Ambrisentan (oral)
Nitric oxide pathwayPhosphodiesterase type 5 inhibitors
 Sildenafil (oral)
 Tadalafil (oral)
Soluble guanylate cyclase stimulator
 Riociguat (oral)