Actively Recruiting

Phase 2
Age: 18Years - 75Years
All Genders
ID06104228

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

Led by Bastiaan Driehuys · Updated on 2026-02-24

20

Participants Needed

1

Research Sites

12 weeks

Total Duration

On this page

Sponsors

B

Bastiaan Driehuys

Lead Sponsor

A

American Heart Association

Collaborating Sponsor

AI-Summary

What this Trial Is About

Researchers are studying pulmonary arterial hypertension (PAH), including its common subtypes idiopathic PAH (IPAH) and connective tissue disease-associated PAH (PAH-CTD). The study aims to develop pulmonary vascular biomarker signatures using hyperpolarized 129Xe MRI to differentiate these subtypes and to evaluate the ability of this imaging to monitor disease progression and treatment response over time. Additional assessments such as laboratory tests, echocardiography, and six-minute walk distance (6MWD) are also used to support these evaluations. The study is observational and enrolls 20 subjects divided into two groups: 10 with IPAH and 10 with PAH-CTD. Participants will undergo hyperpolarized 129Xe MRI and MR spectroscopy at baseline, 3 months, 6 months, and 12 months. Alongside imaging, data from standard clinical assessments including labs, echocardiography, and 6MWD will be collected at these same timepoints to track changes. Participants will be monitored for changes in pulmonary vascular remodeling, red blood cell oscillation amplitude, 6MWD, NTproBNP levels, and WHO functional class over one year. Safety is assessed by tracking adverse events, serious adverse events, withdrawals, and specific examinations such as electrocardiograms and vital signs. The total follow-up period for each participant is one year, with multiple scheduled evaluations to comprehensively assess disease status and response.

CONDITIONS

Brief Title

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

Who Can Participate

Age: 18Years - 75Years
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Age between 18 and 75 years
  • Diagnosis of idiopathic pulmonary arterial hypertension or pulmonary arterial hypertension associated with connective tissue disease
  • WHO functional class 2 or 3
  • Mean pulmonary artery pressure greater than 20 mmHg
  • Pulmonary capillary wedge pressure 15 mmHg or less
  • Pulmonary vascular resistance greater than 2 Wood Units
  • No other cause identified for idiopathic PAH (Arm 1)
  • Diagnosis of connective tissue disease (Arm 2)
Not Eligible

You will not qualify if you...

  • Pulmonary hypertension other than idiopathic PAH or PAH associated with connective tissue disease
  • Any condition that prevents the performance of 129Xe MRI scans

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person)

Diagnostic Evaluation

Duration - 12 months

Participants undergo 129Xe MRI/MRS scans and standard of care assessments to evaluate pulmonary arterial hypertension status.

4 visits (at baseline, 3 months, 6 months, and 12 months)

Trial Site Locations

Total: 1 location

1

Duke University Medical Center

Durham, North Carolina, United States, 27710

Actively Recruiting

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Research Team

C

Claudia Salazar

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NON_RANDOMIZED

Model

SINGLE_GROUP

Primary Purpose

DIAGNOSTIC

Number of Arms

2

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