Congenital Heart Defects, Functional Single Ventricle
Conditions
Keywords
Pulmonary vascular resistance, Heart defects, Congenital, Receptors, endothelin, Bosentan
Brief summary
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
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.
Interventions
DRUGBosentan
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.
Sponsors
Shanghai Jiao Tong University School of Medicine
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
4 Months to 18 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Length of hospital stay and ICU stay | 12 months after Fontan operation |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Symptoms of increased PVR | 12 months after Fontan operation | facial edema plural effusion pericardium effusion |
| WHO functional class | 12 months after Fontan operation | — |
Countries
China
Outcome results
None listed