Table 3:

Currently Approved Medications for Treatment of Pulmonary Arterial

Class	Drug	Route of Administration	Dose
Prostacyclin derivatives	Epoprostenol	IV infusion	2 ng/kg/min Increased as tolerated
	Iloprost	Inhaled	2.5 – 5.0 µg 6–9 inhalations /day
	Treprostinil	Oral	0.25 mg bid or 0.125 mg tid Increase 0.125 mg bid every 3–4 days
		Inhaled	18–54 µg (3–9 inhalations) 4 times daily
		Subcutaneous or IV infusion	1.25 ng/kg/min; increase 1.25 ng/kg/min per week based on clinical response; after week 4 increase by 2.5 ng/kg/min per week 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 IV injection	20 mg every 8 h
	Tadalafil	Oral	40 mg once daily
Soluble cGMP stimulators	Riociguat	Oral	0.5–1.0 mg every 8 h (increase 0.5 mg every 2 wk as tolerated to maximum dose 2.5 mg)
Prostacyclin receptor agonists	Selexipag	Oral	200 mg twice daily Increase as tolerated to maximum dose of 16,000 mg twice daily