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

Who can join

Ages 18 to 75 · All sexes

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.

About the study

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).

What is being tested

• 129Xe Hyperpolarized (drug)

Sponsor: Bastiaan Driehuys · Participants: 20 · Started: Aug 12, 2024

Contact the study team

• Claudia Salazar · Phone: +1 919 660 2026

Official record on ClinicalTrials.gov — NCT06104228

Locations in the U.S.

Conditions

• Pulmonary Arterial Hypertension
• Familial Primary Pulmonary Hypertension
• Connective Tissue Disorders

From ClinicalTrials.gov, data retrieved Sep 29, 2026. Each study sets its own eligibility; the study team decides who can join.