Bosentan Therapy for High Risk Staged Fontan Procedure in Children With Functional Single Ventricle
试验速览
- 阶段
- 2 期
- 状态
- 已完成
- 发起方
- 入组人数
- 34
- 试验地点
- 1
- 主要终点
- Length of hospital stay and ICU stay
研究概览
简要总结
Bosentan is a kind of dual endothelin receptor antagonist.The purpose of this study is to investigate if Bosentan therapy can modify the outcome of children with functional single ventricle.
详细描述
Increased pulmonary vascular resistance (PVR) is a serous issues in children with functional single ventricle during the staged operative period. The purpose of this study is to investigate if Bosentan therapy can improve the survival and life quality after staged Fontan procedure in the children with high risk of increased PVR.
研究设计
- 研究类型
- Interventional
- 分配方式
- Non Randomized
- 干预模型
- Parallel
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 4 Months 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Signed informed consent obtained from patient's legally acceptable representative.
- •Pediatric patients waiting for staged Fontan procedure with high risk of increased PVR after bidirectional cavopulmonary connection (BCPC)
- •Transpulmonary pressure gradiant (TPG) > 10mmHg when the obstruction of anastomosis and lung problem were excluded.
- •With the diagnosis of high risk of increased PVR, such as associated with TAPVC, after pulmonary artery banding, after systemic to pulmonary shunt more than 6 months, and et al.
- •Diagnosed as increased PVR with catheterization.
排除标准
- •PAH associated with conditions other than those mentioned above, e.g., iPAH, PAH secondary to portal hypertension, HIV patient with opportunistic infection
- •Psychotic, addictive or other disorder limiting the ability to provide informed consent or to comply with study requirements
- •AST and/or ALT > 3 times the upper limit of normal ranges.
- •Hemoglobin concentration < 75% the lower limit of normal ranges
- •Treatment or planned treatment with another investigational drug within 3 months of screening
- •Treatment with calcineurin-inhibitors (e.g., cyclosporine A and tacrolimus), fluconazole, glibenclamide (glyburide) within 1 week of enrollment of this study
- •Known hypersensitivity to bosentan or any of the excipients
研究组 & 干预措施
Bosentan group
Bosentan 2mg/kg/dose twice a day and routinely therapy in children with functional single ventricle during the period of staged Fontan procedure with high risk of increased PVR.
干预措施: Bosentan (Drug)
结局指标
主要结局
Length of hospital stay and ICU stay
时间窗: 12 months after Fontan operation
次要结局
- Symptoms of increased PVR(12 months after Fontan operation)
- WHO functional class(12 months after Fontan operation)
研究者
Limin Zhu
Attendant doctor, Department of thoracic and cardiovascular surgery
Shanghai Jiao Tong University School of Medicine