Pulmonary Arterial Hypertension (PAH), Pulmonary Hypertension
Conditions
Brief summary
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.
Interventions
None listed
Sponsors
Franz Rischard, DO
Acceleron Pharma, Inc., a wholly-owned subsidiary of Merck & Co., Inc., Rahway, NJ USA
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 65 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| To assess the reverse remodeling of previously affected vascular structures by using intravascular ultrasound at Baseline, 3 month, 6 month or 9 month. | 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. | This is a medical imaging technique to see inside blood the vessels, especially the arteries around the heart. A catheter with an ultrasound probe at the tip of it will be inserted into a blood vessel and guided to the area that needs to be examined and images of the inside of the vessel will be obtained. |
| To assess the reverse remodeling of previously affected vascular structures by using pulmonary wedge angiography at Baseline, 3 month, 6 month or 9 month. | 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 pulmonary wedge angiography will look at the blood vessels in the lungs to see how blood flows. A catheter is inserted into a vein and it is moved carefully until it reaches the pulmonary artery and temporarily blocks that small vessel. A contrast dye will be injected through the catheter making the blood vessels visible on x-ray images. Several images are captured with the dye (contrast), called an angiogram. |
| To examine the longitudinal effects of sotatercept on ventilation perfusion matching with Ventilation/perfusion single photon emission computed tomography (V/Q SPECT) | 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. | During the V/Q SPECT, participants will breathe in small amounts of radioactive tracers and will receive an injection of another tracer into your vein that flows with your blood into your lungs. The special camera takes pictures of the tracers to see where air and blood flow in your lungs. |
Countries
United States
Contacts
CONTACTThe clinical and translational sciences research center
CONTACTStudy Coordinator
PRINCIPAL_INVESTIGATORFranz Rischard, MD
University of Arizona
Outcome results
None listed