Pulmonary Arterial Hypertension
Conditions
Brief summary
It has been hypothesized that one of the benefits of bosentan relates to pulmonary vascular remodeling. The investigators believe that this study will help document the nature of beneficial changes that occur in patients with Pulmonary Arterial Hypertension (PAH) in response to bosentan therapy. In turn, demonstrating that changes in pulmonary vascular structure and function accompany clinical improvement.
Detailed description
Subjects will have Optical coherence tomography (OCT) and Intravascular Ultrasound (IVUS) imaging during the right heart catheterization that diagnoses them as having PAH. Subjects will start their bosentan and will be followed by monthly clinic visits for 4 months. At 4 months they will have repeat OCT and IVUS imaging.
Interventions
DEVICESt Jude Medical C7 Dragonfly Optical Coherence Tomography (OCT)
All subjects will have OCT and IVUS imaging during their right heart catheterization.
DEVICEBoston Scientific Intravascular Ultrasound
All subjects will have OCT and IVUS imaging during their right heart catheterization.
Sponsors
Actelion
Massachusetts General Hospital
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 75 Years
Healthy volunteers
No
Inclusion criteria
1. WHO GROUP 1 2. AGE 18-75 3. Baseline 6-min walk distance (6MWD) between 200 and 450 m 4. Diagnosed with pulmonary artery hypertension during right heart catheterization (Mean Pulmonary Artery Pressure \> 25mmHg) General
Exclusion criteria
1. Pregnant or nursing 2. Acute or chronic illness other than those associated with PAH (collagen vascular disease, human immunodeficiency virus, or anorexigen use) 3. Previously received any investigational medications, prostanoids, or phosphodiesterase inhibitors 4. eGFR \< 60 5. Angina 6. Syncope 7. Failing right ventricle 8. Hemoptysis
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change in the measurement of Pulmonary arterial intimal + Medial thickening by OCT pre and post treatment with bosentan at 4 months | Baseline and 4 months |
Secondary
| Measure | Time frame |
|---|---|
| Change in pulmonary arterial pulsatility by IVUS pre and post treatment with bosentan at 4 months | Baseline and 4 months |
| Change in pulmonary arterial pressure/elastic strain index by IVUS pre and post treatment with bosentan at 4 months | Baseline and 4 months |
Countries
United States
Outcome results
None listed