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

NCT ID: NCT06104228

Last Updated: 2025-10-21

Basic Information

• Recruitment Status: RECRUITING
• Clinical Phase: PHASE2
• Total Enrollment: 20 participants
• Study Classification: INTERVENTIONAL
• Study Start Date: 2024-08-12
• Study Completion Date: 2026-03-31

Brief Summary

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).

Detailed Description

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

Conditions

Pulmonary Arterial Hypertension; Idiopathic Pulmonary Arterial Hypertension; Pulmonary Arterial Hypertension Associated With Connective Tissue Disease (Disorder); Connective Tissue Diseases

Study Design

• Allocation Method: NON_RANDOMIZED
• Intervention Model: SINGLE_GROUP
• Primary Study Purpose: DIAGNOSTIC
• Blinding Strategy: NONE

Study Groups

Idiopathic Pulmonary Arterial Hypertension

Arm 1... patients with IPAH

Group Type: OTHER

129Xe Hyperpolarized

Intervention Type: DRUG

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

Pulmonary Arterial Hypertension Associated with Connective Tissue Disease

Arm 2... patients with CTD-PAH

Group Type: OTHER

129Xe Hyperpolarized

Intervention Type: DRUG

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

Interventions

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

Intervention Type: DRUG

Eligibility 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.

• Minimum Eligible Age: 18 Years
• Maximum Eligible Age: 75 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

American Heart Association

OTHER

Sponsor Role: collaborator

Bastiaan Driehuys

OTHER

Sponsor Role: lead

Responsible Party

Bastiaan Driehuys

Professor of Radiology

Responsibility Role: SPONSOR_INVESTIGATOR

Principal Investigators

Fawaz Alenezi, MD

Role: PRINCIPAL_INVESTIGATOR

Duke Univeristy

Locations

Duke University Medical Center

Durham, North Carolina, United States

Site Status: RECRUITING

Countries

United States

Central Contacts

Claudia Salazar

Role: CONTACT

claudia.salazar@duke.edu

+1 919 660 2026

Facility Contacts

David Ptashnik

Role: primary

david.ptashnik@duke.edu

9196682642

Other Identifiers

Pro00113893

Identifier Type: -

Identifier Source: org_study_id