Pulmonary HypertensionPhase 2Sox-PHNCT06351345
129 Xenon Imaging in Patients Treated With Sotatercept
A Phase 2 study testing 129Xe Hyperpolarized in people with Pulmonary Hypertension and Pulmonary Arterial Hypertension, run by Bastiaan Driehuys at 1 site in United States.
Trial summary
Determine the ability of 129Xe MRI/MRS biomarker signatures to non-invasively monitor pulmonary vascular reverse remodeling induced by sotatercept in pulmonary arterial hypertension (PAH). Read more about this study
- Who is it for?
- Ages 18 to 75 years
- Conditions the study is for
- Pulmonary Hypertension, Pulmonary Arterial Hypertension
- What is being tested?
- 129Xe Hyperpolarized
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 14 participants
- Where
- 1 siteUnited States
- Study dates
- 27 Oct 2025to 4 Feb 2030
- Phase
- Phase 2
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 to 75 years
- All sexes
- No healthy volunteers
- Adult, Older adult
Who may be able to join
- Outpatients of either gender, Age 18-75
- Diagnosis of precapillary PH (right heart catheterization demonstrating hemodynamic criteria of a mean pulmonary artery pressure (mPAP) ≥ 20 mmHg, pulmonary vascular resistance ≥ 5 WU, pulmonary capillary wedge pressure ≤ 15 mmHg) in the setting of Group 1 (PAH)
- Willing and giving informed consent and adhere to visit/protocol schedules (consent must be given before any study procedures are performed).
- On a stable dose of background PAH therapy for > 90 days prior to study enrollment
- Women of childbearing potential must have a negative urine pregnancy test before MRI
Who cannot join
- Moderate to severe heart disease (LVEF < 45%, Severe LV hypertrophy, Moderate to severe valvular disease)
- Chronic thromboembolic disease
- PH due to schistosomiasis
- Active cancer
- Sickle cell anemia
- Prisoners and pregnant women will not be approached for the study
- Conditions that will prohibit MRI scanning (metal in eye, claustrophobia, inability to lie supine)
- Medical or psychological conditions which, in the opinion of the investigator, might create undue risk to the participant or interfere with the participant's ability to comply with the protocol requirements
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
United States1 site, 1 recruiting
- Recruiting
Duke University Medical Center
Durham, North Carolina, 27710
Claudia Salazar, BS, 919-660-2026, claudia.salazar@duke.edu
Sudarshan Rajagopal, MD, PhD
About this study
Official title: 129Xenon MR Imaging and Spectroscopy Response to Sotatercept in Pulmonary Arterial Hypertension
Determine the ability of 129Xe MRI/MRS biomarker signatures to non-invasively monitor pulmonary vascular reverse remodeling induced by sotatercept in pulmonary arterial hypertension (PAH).
Read the detailed description
The researchers hypothesize that 129Xe MRI/MRS biomarker signatures of pulmonary vascular remodeling will predict short- and long-term response and efficacy to PAH patients who are receiving sotatercept as clinical standard-of-care. At baseline (prior to the treatment with sotatercept), 3, and 12 months of follow-up, the research team will perform 129Xe MRI/MRS scans. 129Xe MRI/MRS metrics, including: (1) 129Xe MRI ventilation defect (reflecting gas exchange abnormalities), (2)129Xe MRI RBC defect percentage (reflecting pulmonary capillary blood volume), (3) 129Xe MRI membrane uptake percentage (reflecting lung interstitial wall thick-ness and inflammation), and (4) 129Xe MRS oscillation amplitude (reflecting degree of pre/post-capillary PH) as well as standard-of-care assessments including labs, echocardiography, NTproBNP and 6MWD will be acquired at each visit. The investigators expect that 129Xe MRI/MRS biomarker signatures will improve prediction of treatment response and clinical outcomes (hospitalizations and death) compared to standard risk assessment with functional class, 6MWD, and NTproBNP.
This study would allow an assessment of sotatercept's role in promoting pulmonary vascular reverse remodeling. It could also improve outcome assessment in clinical trials to a biomarker that is more accurate and precise, thus allowing greater reliability in assessment of treatment effect and allowing smaller clinical trial size. Lastly, three-dimensional functional lung imaging could provide greater individualized assessment of lung function and tailoring of therapy, thus optimizing precision and personalized medicine.
PAH is characterized by obstructive vasculopathy of the pulmonary arterioles that results in right heart failure and death. The pulmonary vascular remodeling in PAH includes neointimal proliferation, medial hypertrophy, plexiform arteriopathy, and fibrosis; these changes can also differ between these subtypes. This results in specific changes in cardiac and pulmonary physiology, most notably: (1) an increase in the pulmonary vascular resistance (PVR) through the blood vessels due to their obstruction; and (2) a decrease in sur-face area and capillary blood volume for gas exchange through disruption of the normal capillary-alveolar interface with a decrease in the diffusion limit for carbon monoxide (DLCO). The increase in PVR results in increased afterload on the right heart, resulting in right ventricular (RV) dysfunction and failure. Similarly, gas exchange abnormalities contribute to decreased ventilatory efficiency and exercise limitation. Current treatments, which target the prostacyclin, endothelin-1, or nitric oxide pathways, slow disease progression. However, these drugs are thought to act largely through vasodilation, and not through remodeling. For that reason, the 5-year survival rate in PAH is still only approximately 60%, highlighting the need for therapies targeting pulmonary vascular remodeling pathways.
- Being tested
- 129Xe Hyperpolarized
- Keywords
- Xenon, MRI
- Medical subject headings
- Hypertension, Pulmonary, Pulmonary Arterial Hypertension
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
129Xe HyperpolarizedDrug
Each xenon dose will be limited to a volume less than 25% of participant lung capacity (TLC).
| Study group | Role | What they receive |
|---|---|---|
| Pulmonary Arterial Hypertension Patients Receving Sotatercept | Other | PAH patients receiving sotatercept as treatment for their PAH Receives: Drug: 129Xe Hyperpolarized |
What is being measured
The primary outcome is the main question the study is designed to answer.
Change in Xenon MRI Red Blood Cell (RBC) percentage
Measured: baseline, 3,and 12 months
Change in cardiogenic oscillation amplitudes
Measured: baseline, 3, and 12 months
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted8 Apr 2024
- Study start27 Oct 2025
- Primary completion4 Feb 2029 (estimated)
- Study completion4 Feb 2030 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Pulmonary HypertensionStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase 2
- Primary purpose
- Diagnosis
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Enrolment
- 14 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Bastiaan Driehuys
- Sponsor type
- University, hospital or other
- Collaborators
- Merck Sharp & Dohme LLC
- Responsible party
- Sponsor-investigator, Bastiaan Driehuys, Duke University
- Investigators
- Sudarshan Rajagopal, MD, PhD, Principal investigator, Duke University
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT06351345
- Sponsor's protocol ID
- Pro00115215
- Acronym
- Sox-PH
- Status last verified
- Feb 2026
- First submitted
- 2 Apr 2024
- Last updated
- 24 Feb 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- Yes
- FDA-regulated device
- No
- Individual data sharing
- No
Study at a glance
From the registry record, last updated by the sponsor on 24 Feb 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- 129Xenon MR Imaging and Spectroscopy Response to Sotatercept in Pulmonary Arterial Hypertension
- ClinicalTrials.gov ID
- NCT06351345
- Acronym / sponsor ID
- Sox-PH / Pro00115215
- Keywords
- Xenon, MRI
- Phase
- Phase 2
- Study type
- Interventional
- Recruitment status
- Recruiting
- Enrolment
- 14 participants (estimated)
- Lead sponsor
- Bastiaan DriehuysUniversity, hospital or other
- Collaborators
- Merck Sharp & Dohme LLC
- Study start
- 27 Oct 2025 (actual)
- Primary completion
- 4 Feb 2029 (estimated)
- Study completion
- 4 Feb 2030 (estimated)
- First posted
- 8 Apr 2024 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Determine the ability of 129Xe MRI/MRS biomarker signatures to non-invasively monitor pulmonary vascular reverse remodeling induced by sotatercept in pulmonary arterial hypertension (PAH).
Detailed description
The researchers hypothesize that 129Xe MRI/MRS biomarker signatures of pulmonary vascular remodeling will predict short- and long-term response and efficacy to PAH patients who are receiving sotatercept as clinical standard-of-care. At baseline (prior to the treatment with sotatercept), 3, and 12 months of follow-up, the research team will perform 129Xe MRI/MRS scans. 129Xe MRI/MRS metrics, including: (1) 129Xe MRI ventilation defect (reflecting gas exchange abnormalities), (2)129Xe MRI RBC defect percentage (reflecting pulmonary capillary blood volume), (3) 129Xe MRI membrane uptake percentage (reflecting lung interstitial wall thick-ness and inflammation), and (4) 129Xe MRS oscillation amplitude (reflecting degree of pre/post-capillary PH) as well as standard-of-care assessments including labs, echocardiography, NTproBNP and 6MWD will be acquired at each visit. The investigators expect that 129Xe MRI/MRS biomarker signatures will improve prediction of treatment response and clinical outcomes (hospitalizations and death) compared to standard risk assessment with functional class, 6MWD, and NTproBNP.
This study would allow an assessment of sotatercept's role in promoting pulmonary vascular reverse remodeling. It could also improve outcome assessment in clinical trials to a biomarker that is more accurate and precise, thus allowing greater reliability in assessment of treatment effect and allowing smaller clinical trial size. Lastly, three-dimensional functional lung imaging could provide greater individualized assessment of lung function and tailoring of therapy, thus optimizing precision and personalized medicine.
PAH is characterized by obstructive vasculopathy of the pulmonary arterioles that results in right heart failure and death. The pulmonary vascular remodeling in PAH includes neointimal proliferation, medial hypertrophy, plexiform arteriopathy, and fibrosis; these changes can also differ between these subtypes. This results in specific changes in cardiac and pulmonary physiology, most notably: (1) an increase in the pulmonary vascular resistance (PVR) through the blood vessels due to their obstruction; and (2) a decrease in sur-face area and capillary blood volume for gas exchange through disruption of the normal capillary-alveolar interface with a decrease in the diffusion limit for carbon monoxide (DLCO). The increase in PVR results in increased afterload on the right heart, resulting in right ventricular (RV) dysfunction and failure. Similarly, gas exchange abnormalities contribute to decreased ventilatory efficiency and exercise limitation. Current treatments, which target the prostacyclin, endothelin-1, or nitric oxide pathways, slow disease progression. However, these drugs are thought to act largely through vasodilation, and not through remodeling. For that reason, the 5-year survival rate in PAH is still only approximately 60%, highlighting the need for therapies targeting pulmonary vascular remodeling pathways.
Study design
- Primary purpose
- Diagnosis
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Pulmonary Arterial Hypertension Patients Receving Sotatercept | Other | Drug: 129Xe HyperpolarizedPAH patients receiving sotatercept as treatment for their PAH |
Who may qualify
- Ages
- 18 to 75 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
Key inclusion criteria (5 listed)
- Outpatients of either gender, Age 18-75
- Diagnosis of precapillary PH (right heart catheterization demonstrating hemodynamic criteria of a mean pulmonary artery pressure (mPAP) ≥ 20 mmHg, pulmonary vascular resistance ≥ 5 WU, pulmonary capillary wedge pressure ≤ 15 mmHg) in the setting of Group 1 (PAH)
- Willing and giving informed consent and adhere to visit/protocol schedules (consent must be given before any study procedures are performed).
- On a stable dose of background PAH therapy for > 90 days prior to study enrollment
- Women of childbearing potential must have a negative urine pregnancy test before MRI
Key exclusion criteria (8 listed)
- Moderate to severe heart disease (LVEF < 45%, Severe LV hypertrophy, Moderate to severe valvular disease)
- Chronic thromboembolic disease
- PH due to schistosomiasis
- Active cancer
- Sickle cell anemia
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| 129Xe HyperpolarizedEach xenon dose will be limited to a volume less than 25% of participant lung capacity (TLC). | Drug | Pulmonary Arterial Hypertension Patients Receving Sotatercept |
Primary outcome
Change in Xenon MRI Red Blood Cell (RBC) percentage
Time frame: baseline, 3,and 12 months
Change in cardiogenic oscillation amplitudes
Time frame: baseline, 3, and 12 months
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 24 Feb 2026.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Sudarshan Rajagopal, MD, PhD principal investigator
Duke University
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A Phase 2 study testing 129Xe Hyperpolarized in people with Pulmonary Hypertension and Pulmonary Arterial Hypertension, run by Bastiaan Driehuys at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 5 inclusion and 8 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 24 Feb 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
What does this phase mean?
Phase 2 studies give the treatment to a larger group to see whether it has the intended effect and to keep checking safety.
Not every study follows the same path, and some studies (observational studies, device studies) have no phase.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Claudia Salazar (+1 919 660 2026, claudia.salazar@duke.edu), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Change in Xenon MRI Red Blood Cell (RBC) percentage (time frame: baseline, 3,and 12 months). The study lists 2 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 24 Feb 2026.