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Table 22 Use of pulmonary arterial hypertension therapies in children

Drug Paediatric study data European Medicines Agency approval

for use in children with PAH Ref.

Phosphodiesterase 5 inhibitors (oral)

Sildenafil RCT, open-label extension: tolerability, efficacy Yes, for ≥1 year of age Recommended dosing: ,20 kg: 30 mg/day in 3 doses; ≥20 kg: 60 mg/ day in 3 doses Avoid higher dosing in children (.3 mg/kg/day) 613,614 Tadalafil RCT, open-label: safety, tolerability, pharmacokinetics No Suggested dosing: 0.5–1 mg/kg/day in one dose Max: 40 mg/day Evaluated only in children aged .3 years 615,616 Endothelin receptor antagonists (oral) Bosentan Open-label, uncontrolled: safety, tolerability, pharmacokinetics, efficacy Yes, for ≥1 year of age Paediatric formulation Recommended dosing: 4 mg/kg/day in 2 doses Max: 250 mg/day 617–620 Ambrisentan Open-label, uncontrolled: safety, tolerability, pharmacokinetics Yes, for children aged .8 years Recommended dosing: 2.5–10 mg/day in one dose 621,622 Macitentan Insufficient data in children Open-label, ongoing: efficacy, safety, pharmacokinetics in children aged 2–18 years No Prostacyclin analogues (i.v./s.c.) Epoprostenol i.v. Cohort studies, retrospective No Suggested dosing: Starting dose: 1–2 ng/kg/min without a known maximum In children, a stable dose is usually 40–80 ng/kg/min Dose increases may be required 623–626 Treprostinil i.v./ s.c. Cohort studies, retrospective: pharmacokinetics No Suggested dosing: Starting dose: 2 ng/kg/min without a known maximum In children, a stable dose is usually 50–100 ng/kg/min Dose increases may be required 624,626,627 Other Iloprost (inhaled) Insufficient data in children Small case series, retrospective No Selexipag (oral) Insufficient data in children Randomized, placebo-controlled, add-on, ongoing: safety, tolerability, pharmacokinetics in children aged 2–18 years No Riociguat (oral) Insufficient data in children Open-label, ongoing: safety, tolerability, pharmacokinetics in children aged 6–18 years No © ESC/ERS 2022 i.v., intravenous; PAH, pulmonary arterial hypertension; RCT, randomized controlled trial; s.c., subcutaneous. Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026