Response to Bosentan in Children with Functional Single Ventricle

  • Zhuoming Xu, Department of cardiovascular surgery, Shanghai Children’s Medical Center, Medical college of Shanghai Jiaotong University, China
  • Limin Zhu, Department of cardiovascular surgery, Shanghai Children’s Medical Center, Medical college of Shanghai Jiaotong University, China
  • Xiaoman Cai, Department of cardiovascular surgery, Shanghai Children’s Medical Center, Medical college of Shanghai Jiaotong University, China
  • Gang Ji, Department of cardiovascular surgery, Shanghai Children's Medical Center, Medical college of Shanghai Jiaotong University, China
  • Jinfen Liu, Department of cardiovascular surgery, Shanghai Children’s Medical Center, Medical college of Shanghai Jiaotong University, China
  • Zhaokang Su, Department of cardiovascular surgery, Shanghai Children’s Medical Center, Medical college of Shanghai Jiaotong University, China

    • Objective: To describe an early experience of treating 14 children with the dual endothelin receptor antagonist bosentan, which is known to be safe and effective in adults with pulmonary hypertension.
    Background: In cavopulmonary anastomoses (Glenn shunt and its variants, Fontan palliation and its variants), even slight increases in pulmonary vascular resistance can significantly lower the transpulmonary blood flow, therefore, leading to circulation failure. Similar unknowns exist regarding potential for benefit of administration of pulmonary vasoactive agents in functional single ventricle (FSV).
    Design: In this prospective, observational study, 14 children with FSV of high-risk factors undergoing cavopulmonary anastamosis received bosentan therapy (Table). The median age was 11 months (range 5 months–15 years).
    Interventions: Bosentan was given as 2-4 mg/kg/day, twice daily. At the cutoff date, children were treated for a mean of 3.4 months (range 2-5 months).
    Main outcome measures: Response to treatment was judged by WHO functional class, six minute walk test, and oxygen saturation.
    Results: Bosentan was well tolerated. WHO class, six minute walk test, and oxygen saturation improved significantly (Figure 1, 2).
    Conclusion: These data suggest that bosentan, an oral endothelin ETA/ETB receptor antagonist, is efficacious for the treatment of high-risk FSV children. Confirmation of these results in a randomized, controlled trial is essential.