Bosentan Therapy in Children With Functional Single Ventricle

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

Official Title
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Bosentan Therapy for High Risk Staged Fontan Procedure in Children With Functional Single Ventricle

Conditions
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Congenital Heart Defects, Functional Single Ventricle

Intervention / Treatment
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* Drug: Bosentan


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: 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. Exclusion Criteria: 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

Ages Eligible for Study
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Minimum Age: 4 Months
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Bosentan group<br>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. | Drug: Bosentan<br>* 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.<br>* Other names: Tracleer;|
| No Intervention: Routinely group<br>Routinely therapy in children with functional single ventricle during the period of staged Fontan procedure with high risk of increased PVR. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Length of hospital stay and ICU stay |  | 12 months after Fontan operation | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Symptoms of increased PVR | facial edema plural effusion pericardium effusion | 12 months after Fontan operation | 
| WHO functional class |  | 12 months after Fontan operation | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Pulmonary vascular resistance, Heart defects, Congenital, Receptors, endothelin, Bosentan