Outcome of bosentan treatment in children with pulmonary arterial hypertension associated with congenital heart disease
LI Ai-jie
GU Hong
ZHANG Chen
WU Bang-jun
GUO Bao-jing
Abstract：Objective To describe the safety and effect of the non-selective endothelin receptor antagonist bosentan used in children with pulmonary arterial hypertension (PAH) associated with unrepaired congenital heart disease (CHD).Methods In this monocentre,open-label,uncontrolled and observational study,pediatric patients ( age ≤ 18 years) with PAH associated with CHD between January 2008 and May 2011 were selected.6-minute walk distance (6MWTD),transcutaneous oxygen saturation ( SpO2 ％ ),New York Heart Association functional class ( NY-HA-FC),hemodynamic parameters and liver function were assessed at baseline,3 months,6 months,1 year and at latest follow-up.Results Totally 23 patients were treated with bosentan.The mean age was (9.1 ± 3.6) years (range 2.1-14.7).The mean treatment time was ( 13.3 ±7.5) months (range 6-31 months).Bosentan improved 6MWTD(n =11) from (458 ± 16)m to (496 ±49) m (P =0.035),SpO2％ (n =23) from 89％ ±6％ to 91％ ± 5％ (P =0.009).All patients received cardiac catherization at baseline,of them 12 patients received that again after 6-month treatment.Bosentan improved Qp/Qs from 0.97 ± 0.38 to 1.16 ± 0.39 ( P =0.076) and decreased PVRi from (20.78 ± 8.84) Woods units/m2 to ( 18.13 ± 7.71 ) Woods units/m2 ( P =0.165 ),Rp/Rs from 1.03 ±0.38 to 0.83 ± 0.29 ( P =0.06).No patient died.Conclusions Bosentan can significantly improve 6MWT,SpO2 ％ and NYHA function class,decrease PVRI and Rp/Rs in children with PAH associated with CHD.It is safe and effective in children with PAH associated with CHD.
Keywords：Congenital heart diseasePulmonary arterial hypertensionBosentanCardiac catherization
Publication Date：2012-01-01
Online Publishing Date：2026-09-14(First online date of this platform, not the publication date of the document)