O-024 Outcome Research In 77 Patients With Pulmonary Arterial Hypertension Receiving Sildenafil: A Double-blind, Randomised Controlled Study

Abstract

Purpose PAH resulting from CHD – a major cause of postoperative morbidity and death. Sildenafil: selective inhibitor of phosphodiesterase-5 - an effective and promising pulmonary vasodilator, with minors reverse effects. Methods This monocentric, randomised placebo-controlled study evaluated the efficacy, safety, tolerability of oral Sildenafil in children with severe PAH secondary congenital shunts (simple (14 patients), mixed (35), complex (28)). 77 PAH patients (35 – repaired shunts, 31 – palliative, 11 inoperable) assigned to placebo or Sildenafil – dose of 1–2 mg/kg/day each 8h: 6–12 months. Sildenafil group - 38 (mean age 19, 9 ± 5, 3 months: 16 boys/22 girls); placebo – 39 (mean age 21, 7 ± 7, 8 months: 22 boys/17 girls). Research protocol: FC NYHA; 6-min walk test; O2 saturation; echocardiography PAPm, myocardial performance index (MPI/Tei index), right cardiac catheterisation – PVRI; questionnaire for adverse reactions was available. Results Sildenafil patients improved FC from 3,16 ± 0, 1–2, 15 ± 0,1 (p 2 saturation (+3,1 ± 0,5%) but placebo (+0,6 ± 0,3%), (p 2 (p 2 (p Conclusions Sildenafil – efficient in treating severe PAH secondary to congenital shunts, but even more effective in children after cardiac surgery. Sildenafil improves FC, effort tolerability, O 2 saturation, RV global function, diminishing PAPm and PVRI comparing with placebo. Sildenafil has good safety, tolerability, favourable impact on life quality – insignificant adverse reactions.