Table 2.

Drugs Associated With Endothelium-Derived Relaxing Factors and Endothelium-Derived Contracting Factors for PH Treatment

Drug	Target and Function	Indications
Macitentan	ETAR/ETBR antagonist	PAH, CTEPH, IPF
Bosentan	ETAR/ETBR antagonist	PAH, CTEPH, IPF
Ambrisentan	ETAR antagonist	PAH, SSc-PH, IPF
Sitaxsentana	ETAR antagonist	PAH
ETRQβ-002b	Vaccine for ETAR	PAH
Beraprost	IP, EP3 receptor agonist	ASO, PAH
Epoprostenol	IP, EP1, EP3 receptor agonist	PAH
Selexipag	IP receptor agonist	PAH
Iloprost	IP, EP1, EP2 receptor agonist	SSc, PAH
Treprostinil	IP, DP1, EP2 receptor agonist	PAH
MRE-269b	Selective IP receptor agonist	PAH
Riociguat	Oral stimulator of sGC	PAH, CTEPH
Cinaciguatb	sGC activator	PPHN
Tadalafil	PDE-5 inhibitor	PAH
Sildenafil	PDE-5 inhibitor	PAH
Vardenafilb	PDE-5 inhibitor	PAH
Inhaled NOc	Vasodilator	PAH, PPHN
Inhaled nitriteb	Vasodilator	PAH
Oral L-Citb	Intermediate for NO synthesis	PAH
Oral L-Argb	Substrates for NO synthesis	PAH
6R-BH4b	Cofactor for eNOS	PAH
Rodatristat ethylb	TPH1 inhibitor	PAH
GSK2586881b	Recombinant human ACE2	PAH

ACE2 = angiotensin-converting enzyme 2; ASO = arteriosclerosis obliterans; BH4 = tetrahydrobiopurine; CTEPH = chronic thromboembolic pulmonary hypertension; DP = prostaglandin D₂ receptor; eNOS = endothelial nitric oxide synthase; EP = prostaglandin E₂ receptor; ETAR = ET_A receptor; ETBR = ET_B receptor; IP = prostaglandin I₂ receptor; IPF = idiopathic pulmonary fibrosis; L-Arg = L-arginine; L-Cit = L-citrulline; PAH = pulmonary arterial hypertension; PDE-5 = phosphodiesterase-5; PH = pulmonary hypertension; PPHN = persistent pulmonary hypertension of the newborn; sGC = soluble guanylate cyclase; SSc = systemic sclerosis; TPH1 = tryptophan hydroxylase 1.

^aSitaxsentan was removed from the market because of liver toxicity.

^bExperimental use only. cInhaled NO is for short-term use or experimental use in patients with PAH.