title: Paediatric pulmonary hypertension
text: |
  Paediatric pulmonary hypertension
  |
  bet | 553/654 | Sana | 03.01.2022 | Hajmi | 1,5 Mb. | | #14803 |
  Paediatric pulmonary hypertension
  STARTS-1 trial performed by Barst et al,1 studied the effectiveness of oral sildenafil in children with pulmonary arterial hypertension. 238 children with a weight ≥ 8 kg were randomised to low-, medium-, or high-dose sildenafil or placebo orally 3 times daily for 16 weeks. The primary comparison was percent change from baseline in peak oxygen consumption for the 3 sildenafil doses combined versus placebo. Percent change in PV˙ O2 for the 3 sildenafil doses combined was only marginally significant; however, PV˙ O2, functional class and haemodynamic improvements with medium and high doses suggest efficacy with these doses.
  STARTS-2 was the extension of the STARTS-1 trial.2 In STARTS-2, sildenafil-treated patients continued STARTS-1 dosing; placebo-treated patients were randomised to 1 of the 3 sildenafil dose groups. Patients requiring additional pulmonary arterial hypertension–specific therapy discontinued study treatment; survival follow-up was attempted. Hazard ratios for mortality were 3.95 (95% confidence interval, 1.46–10.65) for high versus low and 1.92 (95% confidence interval, 0.65–5.65) for medium versus low dose; however, multiple analyses raised uncertainty about the survival/dose relationship. In summary, although children randomised to higher compared with lower sildenafil doses had an unexplained increased mortality, all sildenafil dose groups displayed favourable survival for children with pulmonary arterial hypertension. Combined with STARTS-1 data, the overall profile favoured the medium dose.
  |
  | |