The purpose of this study is assess whether ambrisentan can help people with exercise- induced pulmonary arterial hypertension (EIPAH). The investigators also want to find out if ambrisentan is safe to take without causing excessive side effects.
EIPAH population: These participants may provide a unique window into the pathogenesis of PAH. Our data suggest that these participants may represent an early phase of PAH with an abnormal vascular response. The study includes an assessment of the potential impact of ambrisentan on the exercise capacity Advanced Level-3 cardiopulmonary exercise test (CPET) and the World Health Organization functional class (WHO FC).
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
30
5 mg orally every day for 4 weeks. At Week 4, if ambrisentan 5 mg was tolerated, the dose was increased to 10 mg every day for the remainder of the 24-week period.
Brigham and Women's Hospital
Boston, Massachusetts, United States
Changes From Baseline in Peak Exercise Mean Pulmonary Artery Pressure (mPAP), Transpulmonary Pressure Gradient (TPG), and Pulmonary Capillary Wedge Pressure (PCWP)
mPAP is measure of the blood pressure found in the main artery of the lung. TPG is the difference between mean pulmonary arterial pressure and left atrial pressure. PCWP is the pressure measured by wedging a pulmonary catheter with an inflated balloon into a small pulmonary arterial branch.
Time frame: Baseline to Week 24
Change From Baseline in Peak Exercise Pulmonary Vascular Resistance (PVR)
PVR is the resistance offered by the pulmonary circulatory system.
Time frame: Baseline to Week 24
Change From Baseline in Peak Exercise Pulmonary Vascular Compliance (PVC )
PVC is a measure of a pulmonary vein's ability to expand.
Time frame: Baseline to Week 24
Change From Baseline in Peak Exercise Cardiac Output (CO)
CO is the amount of blood pumped by the heart per minute.
Time frame: Baseline to Week 24
Change From Baseline in Peak Exercise Maximum Oxygen Uptake (VO2max)
VO2max is the measurement of the maximum amount of oxygen that an individual can utilize during intense or maximal exercise.
Time frame: Baseline to Week 24
Change From Baseline in 6-minute Walk Distance (6MWD)
6MWD is the distance walked by the participant in 6 minutes.
Time frame: Baseline to Week 24
Borg Dyspnea Scale Score
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The Borg Dyspnea Scale measures how breathless the participant feels. Scores range from 0 (no shortness of breath) to 10 (more short of breath than ever experienced). (minimum score 0, maximum score 10 with 10 being worsening outcome)
Time frame: Baseline and Week 24
World Health Organization Functional Class (WHO FC)
The WHO FC categorizes cardiac disability using four classes, ranging from Class 1 (without limitation of physical activity) to Class 4 (inability to carry on any physical activity without discomfort).
Time frame: Baseline and Week 24