Table 1

FDA approved pharmacological classes for treatment of PAH.

Class	Drug	Rout of administration	Dose
Prostacyclin derivatives	Epoprostenol	IV	Initial dose of 2 ng kg^-1min^-1 Iv infusion, titrated by 1–2 ng kg^-1min^-1q 15 min if tolerated
	Iloprost	Inhaled	2.5 μg inhaled, if tolerated then 5 μg, 6–9 times a day PRN; Maintenance: 2.5–5 μg dose^-1 (max: 45 μg daily)
	Treprostinil	PO	PO: 0.125 mg TID or 0.25 mg BID, titrated by 0.125 mg TID every 3–4 days
		Continuous IV or SC infusion	IV or SC infusion: 1.25 ng kg^-1min^-1 titrated by no more than 1.25 ng kg^-1min^-1per wk based on clinical response; after 4 wk, titrated by no more than 2.5 ng kg^-1min^-1 per wk based on clinical response
Endothelin receptor antagonists	Bosentan	Oral	125 mg twice daily
	Ambrisentan	Oral	5 or 10 mg once daily
	Macitentan	Oral	10 mg once daily
Phosphodiesterase type-5 inhibitors	Sildenafil	Oral	20 mg TID
		IV Injection
	Tadalafil	Oral	40 mg once daily
Soluble cGMP stimulators	Riociguat	Oral	0.5–1.0 mg TID (titrated by 0.5 mg every 2 wk as tolerated to maximum dose 2.5 mg)
Prostacyclin receptor agonists	Selexipag	Oral	200 mg twice daily, titrated as tolerated to maximum dose of 16,000 mg twice daily

Abbreviations: cGMP, Cyclic guanosine monophosphate; FDA, Food and Drug Administration; h, hour; IV, Intravenous; PAH, pulmonary arterial hypertension; SC, subcutaneous.

Blood Res 2021;56:229~242 https://doi.org/10.5045/br.2021.2021117