Outcome of bosentan in treatment in children patients with eisenmenger's syndrome
ZHANG Hong-wei
GU Hong
Abstract：Objective To observe the outcome of children patients with eisenmenger syndrome (ES) treated by bosentan.Methods Twelve children patients (age ≤ 18 years)with ES were selected between June 2008 and February 2012.6-minute walk test distance(6MWTD),transcutaneous oxygen saturation(SpO2),new york heart association (NYHA) heart functional class,hemodynamic parameters mean pulmonary arterial pressure (mPAP),pulmonary arterial resistance index (PVRI),systemic blood flow ratio (Qp/Qs),pulmonary/systemic vascular resistance and pulmonary ratio (Rp/Rs) and liver function were assessed before and after bosentan treatment.Results After the treatment,6MWTD improved from (429 ± 104) m to (475 ± 88) m ; SpO2 from (87 ± 4) ％ to (89 ± 3)％.NYHA functional class stayed stable.All patients received cardiac catherization at baseline,among whom 15 patients received the second cycle of treatment several months later.Bosentan increased Qp/Qs from (0.9 ±0.3) to (1.0 ±0.3),and decreased mPAP from (75 ± 10)mm Hg (1 mm Hg =0.133 kPa) to(74 ± 11)mm Hg,PVRI from (23 ± 10) Wood * m2 to (20 ± 6) Wood * m2,Rp/Rs from (1.1 ± 0.4) to (0.9 ± 0.3),but without statistical significance.All patients could tolerate the treatment.Conclusions Bosentan can significantly improve 6MWTD,SpO2.Meanwhile the bosentan treatment is safe in these ES oatients.
Keywords：Congenital heart diseasePulmonary arterial hypertensionEisenmenger syndromeBosentanChildren
Publication Date：2013-04-30
Online Publishing Date：2026-09-14(First online date of this platform, not the publication date of the document)
Pages：2( 459-460 )