Pulmonary arterial hypertension (PAH)

PAH, adults — start 5 mg once daily (with or without tadalafil); up to 10 mg

PAH, over 12 years — 62.5 mg twice daily (first 4 weeks; continue this dose if under 40 kg)

PAH, over 12 years and over 40 kg — maintenance 125 mg twice daily after 4 weeks

PAH, 12 years and under — twice daily by weight: 4–8 kg: 16 mg; 8–16: 32; 16–24: 48; 24–40: 64 mg

PAH (WHO group 1) — continuous IV infusion via central catheter; start 2 ng/kg/min

PAH, adults — 10 mg once daily

PAH and CTEPH, adults — start 1 mg three times daily; increase by 0.5 mg every 2 weeks to max 2.5 mg three times daily

PAH (WHO group 1), adults — 20 mg three times daily

PAH, children 1–17 years — ≤20 kg: 10 mg; >20 kg: 20 mg three times daily

PAH (WHO group 1, NYHA II–IV) — continuous SC (preferred) or central IV infusion; start 1.25 ng/kg/min