Summary of drugs used to treat pulmonary artery hypertension
| Drug | Licence | Doses used in paediatric studies |
|---|---|---|
| *Licence in the USA and Europe. Not yet licensed in the UK. | ||
| NA, not available; NYHA, New York Heart Association; PAH, pulmonary artery hypertension; PPH, primary pulmonary hypertension. | ||
| Epoprostenol | Treatment of patients with PPH in NYHA class III or IV who have not responded to conventional treatment. Administered by continuous iv infusion. Limited published information on use in children | 2 ng/kg/min initially. Continuous infusion via central line. Doses increase depending on clinical course and tolerance. At 1 year, doses may be up to 50–80 ng/kg/min for some children.6 Higher doses have been used beyond 1 year8 |
| Iloprost | Treatment of adults with PPH in functional class III by inhalation. Currently no published experience in children and adolescents is available. | NA |
| Treprostinil | Treatment of PAH in patients with class II–IV symptoms by continuous subcutaneous infusion. Safety and efficacy in paediatric patients has not been established* | NA |
| Beraprost sodium | Orphan drug. Limited studies in children. Use is experimental | NA |
| Bosentan | Treatment of PPH and PAH secondary to scleroderma in patients with grade III functional status. Safety and efficacy in patients <12 years old has not been substantially documented | 10–20 kg: 31.25 mg daily for 4 weeks then increase to 32.5 mg twice a day |
| 20–40 kg: 31.25 mg twice a day for 4 weeks then increase to 62.5 mg twice a day | ||
| >40 kg: 62.5 mg twice a day for 4 weeks then increase to 125 mg twice a day | ||
| Sildenafil | Not licensed for PPH or PAH. Use is experimental | NA |