The goal of this study is to learn more about how sotatercept works and if it helps the lung arteries become healthier. Sotatercept will be associated with the following: 1. Improvement in capillary blush, reduce the tapering and tortuosity of affected vessels on pulmonary wedge angiography and decreased wall thickness on intravascular ultrasound in previously affected areas. 2. Improvement in previously poorly or non-perfused areas rather than increased perfusion to previously perfused areas. 3. No changes in baseline ventilation and improvement and ventilation/perfusion matching.
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To assess the reverse remodeling of previously affected vascular structures by using intravascular ultrasound at Baseline, 3 month, 6 month or 9 month.
Timeframe: At Baseline and 6 month Evaluation for phase 1 feasibility group and Phase 2 prevalent group. At Baseline, 3 month evaluation and 9 month Evaluation for phase 2 treatment-naive group.
To assess the reverse remodeling of previously affected vascular structures by using pulmonary wedge angiography at Baseline, 3 month, 6 month or 9 month.
Timeframe: At Baseline and 6 month Evaluation for phase 1 feasibility group and Phase 2 prevalent group. At Baseline, 3 month evaluation and 9 month Evaluation for phase 2 treatment-naive group.
To examine the longitudinal effects of sotatercept on ventilation perfusion matching with Ventilation/perfusion single photon emission computed tomography (V/Q SPECT)
Timeframe: At Baseline and 6 month Evaluation for phase 1 feasibility group and Phase 2 prevalent group. At Baseline, 3 month evaluation and 9 month Evaluation for phase 2 treatment-naive group.
The clinical and translational sciences research center