title: [Efficacy of endothelin receptor antagonist bosentan on the long-term prognosis in patients after Fontan operation].
abstract: OBJECTIVE
  To investigate the long-term effect of bosentan on outcome in patients after Fontan operation.
  
  
  METHODS
  Patients after Fontan surgery were randomly divided into bosentan group (B, n = 16) and control group (C, n = 23). Bosentan was applied within 7 days after Fontan surgery as follows: at the first month, 7.8125 mg Bid for patients with body weight ≤ 10 kg; 15.625 mg Bid for patients with body weight between 10-20 kg; 31.25 mg Bid for patients with body weight 20-30 kg and 62.5 mg Bid for patients with body weight > 30 kg. At the second month, the bosentan dose was doubled and Bosentan therapy was continued for more than 1 year. Group C didn't take drugs affecting pulmonary artery pressure. All patients were followed up for 2 years and incidence of mortality, protein losing enteropathy, pulmonary arteriovenous fistulae, 6-minute walk test, heart function were compared between the two groups.
  
  
  RESULTS
  After 2 years, mortality tended to be lower in group B compared to group C [6.25% (1/16) vs. 21.8% (5/23), P > 0.05]. Incidence of pulmonary arteriovenous fistulae and protein losing enteropathy were significantly lower in group B than in group C (6.25% vs. 34.78%, P = 0.01;6.25% vs. 39.13%, P = 0.02, respectively) . The results of 6-minute walk test[ (485 ± 44) m vs. (302 ± 183) m] and heart function in group B (3 NYHA III/IV patients in group B vs. 14 NYHA III/IV patients in group C, all P < 0.05) were all better than group C. The concentrations of vasoactive factors such as brain natriuretic peptide (BNP, 279.07 ± 128.17 vs. 457.67 ± 221.30), endothelin (ET, 3.30 ± 0.61 vs. 4.98 ± 1.24) and thromboxane (TXA2, 97.2 ± 24.0 vs. 163.22 ± 24.4) were also significantly lower in group B than in group C (all P < 0.05). Prostacyclin (PGI2) level and incidence of arrhythmias were similar between the two groups. There was no thrombotic event in both groups during follow up.
  
  
  CONCLUSIONS
  Bosentan trerapy in patients post Fontan operation could reduce the incidence of pulmonary arteriovenous fistulae and protein losing enteropathy and improve heart function.
authors: Wuhan Asia Heart Hospital Cardiac Surgery, Wuhan 430022, China.
authors: Xiao-ke
authors: XK
authors: Shang
authors: Wuhan Asia Heart Hospital Cardiac Surgery, Wuhan 430022, China.
authors: Yan-ping
authors: YP
authors: Li
authors: Wuhan Asia Heart Hospital Cardiac Surgery, Wuhan 430022, China.
authors: Mei
authors: M
authors: Liu
authors: Wuhan Asia Heart Hospital Cardiac Surgery, Wuhan 430022, China.
authors: Hong-mei
authors: HM
authors: Zhou
authors: Wuhan Asia Heart Hospital Cardiac Surgery, Wuhan 430022, China.
authors: Ting
authors: T
authors: Peng
authors: Wuhan Asia Heart Hospital Cardiac Surgery, Wuhan 430022, China.
authors: Xiao-xian
authors: XX
authors: Deng
authors: Wuhan Asia Heart Hospital Cardiac Surgery, Wuhan 430022, China.
authors: Liang
authors: L
authors: Tao
authors: Wuhan Asia Heart Hospital Cardiac Surgery, Wuhan 430022, China. Email: zhanggangchen@medmail.com.cn.
authors: Gang-cheng
authors: GC
authors: Zhang
mesh_terms: D000293:Adolescent; D000077300:Bosentan; D002648:Child; D002675:Child, Preschool; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use*; D005260:Female; D018729:Fontan Procedure; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008297:Male; D011379:Prognosis; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan