Reverse Remodeling of the Pulmonary Vasculature: a Longitudinal, Investigational Study of the Effects of Sotatercept.

Reverse Remodeling of the Pulmonary Vasculature: a Longitudinal, Investigational Study of the Effects of Sotatercept.

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.

Inclusion Criteria - Feasibility Group:

• Group 1 Pulmonary Hypertension (G1PH)-must be idiopathic, drug induced, or hereditary (D/H/I)
• Age < 65 years at time of diagnosis
• Must be 18 years of age or older

Inclusion Criteria - "Real-world" treatment naïve cohort:

• Group 1 Pulmonary Hypertension (G1PH)-all types
• Must be treatment naïve
• Must be 18 years of age or older

Inclusion Criteria - "Real-world" prevalent cohort:

• Group 1 Pulmonary Hypertension (G1PH)-all types
• Must be 18 years of age or older

Exclusion Criteria:

• Contraindication to right heart catheterization (per PI)
• Prior exposure to sotatercept
• Hemoglobin > 18 g/dL at screening
• Estimated GFR < 30 mL/min at screening
• Platelet count <30 at screening
• Positive pregnancy test