129 Xenon MRI as a Biomarker for Diagnosis and Response to Therapy in Pulmonary Arterial Hypertension (PAH)

Study Purpose

The overall study objectives outlined in this study are to derive 129Xe MRI pulmonary vascular biomarker signatures that differentiate common subtypes of PAH and to determine the ability of 129Xe MRI to longitudinally monitor disease progression and response to therapy in PAH, with the aid of additional assessments, such as labs, echocardiography, and six-minute walk distance (6MWD).

Recruitment Criteria

Accepts Healthy Volunteers Healthy volunteers are participants who do not have a disease or condition, or related conditions or symptoms	No
Study Type An interventional clinical study is where participants are assigned to receive one or more interventions (or no intervention) so that researchers can evaluate the effects of the interventions on biomedical or health-related outcomes. An observational clinical study is where participants identified as belonging to study groups are assessed for biomedical or health outcomes. Searching Both is inclusive of interventional and observational studies.	Interventional
Eligible Ages	18 Years - 75 Years
Gender	All

More Inclusion & Exclusion Criteria

Inclusion Criteria:

Arm 1 -IPAH.

- Age: 18-75 years.

- WHO functional class 2 or 3.

- Mean pulmonary artery pressures > 20 mmHg.

- Pulmonary capillary wedge pressure ≤15 mmHg.

- Pulmonary vascular resistance > 2 Wood Units (WU) - No other cause identified for PAH.

Arm 2 -PAH-CTD.

- Age: 18-75 years.

- WHO functional class (FC) 2 or 3.

- Mean pulmonary artery pressures > 20 mmHg.

- Pulmonary capillary wedge pressure ≤15 mmHg.

- Pulmonary vascular resistance > 2 WU.

- Diagnosis of connective tissue disease.

Exclusion Criteria:

- PH other than Idiopathic PAH or PAH associated with CTD; any conditions that prevent the performance of 129Xe MRI scans will be excluded from the study.

Trial Details

Trial ID: This trial id was obtained from ClinicalTrials.gov, a service of the U.S. National Institutes of Health, providing information on publicly and privately supported clinical studies of human participants with locations in all 50 States and in 196 countries.	NCT06104228
Phase Phase 1: Studies that emphasize safety and how the drug is metabolized and excreted in humans. Phase 2: Studies that gather preliminary data on effectiveness (whether the drug works in people who have a certain disease or condition) and additional safety data. Phase 3: Studies that gather more information about safety and effectiveness by studying different populations and different dosages and by using the drug in combination with other drugs. Phase 4: Studies occurring after FDA has approved a drug for marketing, efficacy, or optimal use.	Phase 2
Lead Sponsor The sponsor is the organization or person who oversees the clinical study and is responsible for analyzing the study data.	Bastiaan Driehuys
Principal Investigator The person who is responsible for the scientific and technical direction of the entire clinical study.	Fawaz Alenezi, MD
Principal Investigator Affiliation	Duke Univeristy
Agency Class Category of organization(s) involved as sponsor (and collaborator) supporting the trial.	Other
Overall Status	Not yet recruiting
Countries	United States
Conditions The disease, disorder, syndrome, illness, or injury that is being studied.	Pulmonary Arterial Hypertension, Idiopathic Pulmonary Arterial Hypertension, Pulmonary Arterial Hypertension Associated With Connective Tissue Disease (Disorder), Connective Tissue Diseases

Additional Details

Subject Enrollment This study will consent and enroll 20 subjects total. • For Arm 1, 10 subjects with Idiopathic Pulmonary Arterial Hypertension (IPAH) will be consented and enrolled. For Arm 2, 10 subjects with Connective Tissue Disease Associated Pulmonary Arterial Hypertension (PAH-CTD) will be consented and enrolled. Study Design This study will be observational. Subjects in both arms of the trial will undergo a 129Xe MRI/MRS at timepoints of baseline, 3 months, 6 months, and 12 months. In addition to the this, data from standard of care assessments, such as labs, echocardiography, and six-minute walk distance (6MWD), will also collected at these timepoints. Primary Study Endpoints The primary endpoint for this trial will be the change in defect + low percentage of RBC signal on hyperpolarized 129Xe MRI from baseline to 12 months. Secondary Study Endpoints. There will be several secondary endpoints for this trial:

- Change in regional and global RBC Oscillation Amplitudes on hyperpolarized 129Xe MR spectroscopy from baseline to 12 months.

- Change in 6MWD from baseline to 12 months.

- Change in NTproBNP from baseline to 12 months.

- Change in WHO FC from baseline to 12 months.

Primary Safety Endpoints. There will be several primary safety endpoints for this trial:

- Frequency of Adverse Events (AE) and/or Serious Adverse Events (SAE) - Withdrawals due to adverse event or death.

- Incidence of Adverse Events of Significant Interest (AESI): - Electrocardiogram and any findings.

- Physical examination and vital signs

Arms & Interventions

Arms

Other: Idiopathic Pulmonary Arterial Hypertension

Arm 1... patients with IPAH

Other: Pulmonary Arterial Hypertension Associated with Connective Tissue Disease

Arm 2... patients with CTD-PAH

Interventions

Drug: - 129Xe Hyperpolarized

Each xenon dose will be limited to a volume less than 25% of a subject's total lung capacity (TLC), as is the case for all protocols currently carried out under IND 109490

Contact a Trial Team

If you are interested in learning more about this trial, find the trial site nearest to your location and contact the site coordinator via email or phone. We also strongly recommend that you consult with your healthcare provider about the trials that may interest you and refer to our terms of service below.

Duke University Medical Center, Durham, North Carolina

Status

Address

Duke University Medical Center

Durham, North Carolina, 27710

Site Contact

David Ptashnik

david.ptashnik@duke.edu

919-668-2642