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

NCT06104228 | Recruiting Phase 2 FDA Drug

• 1 other identifier: Pro00113893
• Study Type: interventional
• Target: 20
• Locations: 1 country
• Sites: 1

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).

Conditions

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

Outcome Measures

Primary Outcomes (1)

• Pulmonary Vascular Remodeling

  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

  1 year

Secondary Outcomes (4)

• RBC Oscillation Amplitude

  1 year

• 6 Minute Walk Distance

  1 Year

• NTproBNP

  1 year

• World Health Organization (WHO) Functional Class (FC)

  1 year

Study Arms (2)

Idiopathic Pulmonary Arterial Hypertension

OTHER

Arm 1... patients with IPAH

Drug: 129Xe Hyperpolarized

Pulmonary Arterial Hypertension Associated with Connective Tissue Disease

OTHER

Arm 2... patients with CTD-PAH

Drug: 129Xe Hyperpolarized

Interventions

129Xe Hyperpolarized 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

Idiopathic Pulmonary Arterial Hypertension; Pulmonary Arterial Hypertension Associated with Connective Tissue Disease

Eligibility Criteria

• Age: 18 Years - 75 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• 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

You may not qualify if:

• 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.

Sponsors & Collaborators

• Bastiaan Driehuys: lead
• American Heart Association: collaborator

Study Sites (1)

Duke University Medical Center

Durham, North Carolina, 27710, United States

RECRUITING

MeSH Terms

Conditions

Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Connective Tissue Diseases

Condition Hierarchy (Ancestors)

Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases; Skin and Connective Tissue Diseases

Study Officials

• Fawaz Alenezi, MD

  Duke Univeristy

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Claudia Salazar

CONTACT

+1 919 660 2026; claudia.salazar@duke.edu

Study Design

• Study Type: interventional
• Phase: phase 2
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: DIAGNOSTIC
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Professor of Radiology

Study Record Dates

• First Submitted: October 23, 2023
• First Posted: October 27, 2023
• Study Start: August 12, 2024
• Primary Completion (Estimated): January 1, 2027
• Study Completion (Estimated): March 31, 2027
• Last Updated: February 24, 2026

Record last verified: 2026-02

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)