Pulmonary Arterial Hypertension
Conditions
Brief summary
6 months therapy of Bosentan, an endothelin antagonist, will lead to improvement in pulmonary microvascular endothelial function.
Interventions
DRUGBosentan
62.5 mg Bosentan twice a day for 1 month 125 mg Bosentan twice a day for 5 months
Sponsors
Prof David S Celermajer
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
* Pulmonary arterial hypertension; idiopathic and connective tissue disease associated * Confirmed or invasive haemodynamic: * Mean pulmonary arterial pressure greater than or equal to 25 millimeters of mercury * Pulmonary capillary wedge pressure less than 15 millimeters of mercury * No prior pulmonary hypertension specific therapy * Ability to provide informed consent
Exclusion criteria
* Contra-indications to medications used to test endothelial function; acetylcholine, sodium nitroprusside, NG-Monomethyl-L-Arginine, L-arginine * Advanced renal disease * Previous allergic reaction to contrast agents
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Acetylcholine Vascular Reactivity Response | Baseline and 6 months | Percent pulmonary flow change from baseline after acetylcholine |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Intravascular Ultrasound - Pulmonary Artery Wall Thickness | baseline and 6 months | Change in intima-media thickness |
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Bosentan 62.5 mg Bosentan b.i.d. for 1 month 125 mg Bosentan b.i.d. for 5 months | 8 |
| Total | 8 |
Baseline characteristics
| Characteristic | Bosentan |
|---|---|
| Age, Categorical <=18 years | 0 Participants |
| Age, Categorical >=65 years | 3 Participants |
| Age, Categorical Between 18 and 65 years | 5 Participants |
| Age, Continuous | 66 years STANDARD_DEVIATION 4 |
| Region of Enrollment Australia | 8 participants |
| Sex: Female, Male Female | 5 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 | 0 / 8 |
| serious Total, serious adverse events | 0 / 8 |
Outcome results
Primary
Acetylcholine Vascular Reactivity Response
Percent pulmonary flow change from baseline after acetylcholine
Time frame: Baseline and 6 months
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Bosentan | Acetylcholine Vascular Reactivity Response | 180 percentage of baseline | Standard Deviation 20 |
Secondary
Intravascular Ultrasound - Pulmonary Artery Wall Thickness
Change in intima-media thickness
Time frame: baseline and 6 months
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Bosentan | Intravascular Ultrasound - Pulmonary Artery Wall Thickness | 34 percentage of baseline | Standard Deviation 2 |