Outcome of Bosentan treatment in adult patients with Eisenmenger's syndrome
GU Hong
LI Ai-jie
ZHANG Chen
WANG Xiao-feng
ZHANG Hong-wei
CHENG Meng-pei
WU Bang-jun
GUO Bao-jing
Abstract：Objective To observe the outcome of adult patients with Eisenmenger's syndrome( ES)treated by Bosentan which is known to be safe and effective in patients with idiopathic pulmonary hypertension (IPAH).Methods In this monocentre,open-label,uncontroiled,observational study,aduh patients( age≥18 years)with ES were selected between Janurary 2007 and December 2011.6-minute walk distance(6MWTD),transcutaneous oxygen saturation( SpO2 ),New York Heart Association functional class ( NYHA-FC ),hemodynamic parameters and liver function were assessed at baseline,3 months,6 months,1 year and latest follow-up.Results Totally 8 patients were treated with bosentan.The mean age was(23 ±4) years(range 18.8 ～ 31.1 ).The mean treatment time was (20 ±18)months(range 6-60 months).Bosentan improved 6MWTD(n =8) from(435 ±- 121 ) m to(491 ±92)m (P =0.016) ;SpO2％ (n =8) from(87 ±3)％ to(88 ±5)％ (P =0.140).New York Heart Association functional class stayed stable or improved ( P =0.034).Seven patients received cardiac catherization at baseline,of them 4 patients had a repeated therapy after 6-month.Bosentan improved Qp/Qs and decreased PVRi and Rp/Rs but without a statistical significance.All patients could tolerate the treatment,only one female patient underwent temporary lower gastrointestinal hemorrhage.Conclusions Bosentan caused significant improvement of 6MWTD,SpO2,NYHA function class in patients with ES.Meanwhile bosentan treatment is safe in adult ES patients.
Keywords：Congenital heart diseasePulmonary arterial hypertensionEisenmenger's syndromeBosentanAdult
Publication Date：2012-01-01
Online Publishing Date：2026-09-14(First online date of this platform, not the publication date of the document)