Congenital Heart Disease
Conditions
Keywords
Single ventricle congenital heart disease
Brief summary
Recent studies have shown beneficial effects of sildenafil in patients with single ventricle congenital heart disease. The purpose of this study is to determine whether Bosentan, a drug with similar effects, will have similar benefit in this patient population.
Detailed description
Patients enrolled in the study will take Bosentan. Baseline and follow up measurements will be collected then analyzed.
Interventions
DRUGBosentan
Bosentan 62.5 mg tablet taken orally twice daily for one month, followed by Bosentan 125 mg tablet taken orally twice daily for three months.
Sponsors
Actelion
University of California, Los Angeles
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
SUPPORTIVE_CARE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
12 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* At least 12 years of age * Single ventricle congenital heart disease * Require cardiac magnetic resonance imaging (MRI) for clinical purposes.
Exclusion criteria
* \< 12 years of age * Pregnancy or women who may become pregnant (sexually active and unwilling to use birth control) * Patients taking cyclosporin or glyburide * Patients with baseline liver dysfunction (aspartate aminotransferase or alanine aminotransferase \> 3 times upper limit of normal) * Contraindications to MRI (including pacemakers) * Use of Bosentan within one month prior to enrollment
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Pulmonary Blood Flow as Determined by MRI Velocity Encoding at 3-6 Months | 3-6 months | Magnetic resonance imaging-derived aortic flow |
Countries
United States
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Bosentan Bosentan: Bosentan 62.5 mg tablet taken orally twice daily for one month, followed by Bosentan 125 mg tablet taken orally twice daily for three months. | 10 |
| Total | 10 |
Baseline characteristics
| Characteristic | Bosentan |
|---|---|
| Age, Categorical <=18 years | 0 Participants |
| Age, Categorical >=65 years | 0 Participants |
| Age, Categorical Between 18 and 65 years | 10 Participants |
| Age, Continuous | 34 years STANDARD_DEVIATION 10.9 |
| Region of Enrollment United States | 10 participants |
| Sex: Female, Male Female | 7 Participants |
| Sex: Female, Male Male | 3 Participants |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | — / — |
| other Total, other adverse events | 3 / 10 |
| serious Total, serious adverse events | 0 / 10 |
Outcome results
Primary
Pulmonary Blood Flow as Determined by MRI Velocity Encoding at 3-6 Months
Magnetic resonance imaging-derived aortic flow
Time frame: 3-6 months
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Bosentan | Pulmonary Blood Flow as Determined by MRI Velocity Encoding at 3-6 Months | BaselineAortic Flow Volume | 3.3 L/min | Standard Deviation 1.27 |
| Bosentan | Pulmonary Blood Flow as Determined by MRI Velocity Encoding at 3-6 Months | 3-6 Month Aortic Flow Volume | 4.4 L/min | Standard Deviation 0.9 |
p-value: 0.03t-test, 2 sided