Identifying lung disease signatures using advanced MRI techniques

Defining a Noninvasive Signature for Pulmonary Vascular Remodeling in Group 3 PH

This study is testing a new MRI technique to see if it can help identify which patients with pulmonary hypertension might benefit from specific treatments.

Quick facts

• Phase: PHASE2
• Study type: Interventional
• Enrollment: 30 (estimated)
• Ages: 18 Years and up
• Sex: All
• Sponsor: Duke University (other)
• Locations: 1 site (Durham, North Carolina)
• Trial ID: NCT04778046 on ClinicalTrials.gov

What this trial studies

This study aims to develop a noninvasive signature for pulmonary vascular remodeling in patients with Group 3 pulmonary hypertension (PH) using hyperpolarized 129Xe magnetic resonance imaging (129Xe MRI). The research will compare these MRI signatures with lung explant tissue from patients who have undergone lung transplants to establish a pathologic 'ground truth'. The goal is to identify which patients may respond to pulmonary arterial hypertension (PAH)-specific therapies based on their unique MRI signatures. This approach builds on previous findings that have shown distinct MRI signatures in patients treated with Tyvaso, depending on their underlying lung function.

Who should consider this trial

Good fit: Ideal candidates include adults aged 18 and older who are on the lung transplant waiting list and have a diagnosis of Group 3 pulmonary hypertension.

Not a fit: Patients with moderate to severe heart disease, active cancer, or other specific exclusions such as sarcoidosis or sickle cell anemia may not benefit from this study.

Why it matters

Potential benefit: If successful, this study could lead to improved identification of patients who are likely to benefit from PAH-specific therapies, enhancing treatment outcomes.

How similar studies have performed: Previous studies have shown promising results with similar MRI techniques in identifying pulmonary vascular remodeling, suggesting potential for success in this novel approach.

Eligibility criteria

Inclusion Criteria:

1. Age ≥ 18 years' old
2. Be on the lung transplant waiting list at Duke University Medical Center.
3. PH as defined by RHC - mPAP \> 20 mmHg, PVR \> 3 WU, PCWP \< 15 mmHg
4. Groups defined as:

   PAH: Clinical diagnosis of PAH (Group 1 PH) in the absence of severe chronic lung disease, left heart disease, chronic thromboembolism, sarcoidosis, sickle cell disease, or other causes of non-Group 1 PH.

   COPD-noPH: Clinical diagnosis of COPD in the absence of precapillary PH.

   COPD-PH: Clinical diagnosis of COPD with precapillary PH.

   IPF-noPH: Clinical diagnosis of IPF in the absence of precapillary PH.

   IPF-PH: Clinical diagnosis of IPF with precapillary PH.
5. Willing and able to give informed consent and adhere to visit/protocol schedules (consent must be given before any study procedures are performed).

Exclusion Criteria:

1. Moderate to severe heart disease (LVEF \< 45% or severe LV Hypertrophy).
2. Sarcoidosis.
3. Active cancer.
4. Sickle cell anemia.
5. Liver disease (Childs-Pugh class C).
6. Prisoners and pregnant women will not be approached for the study.
7. Inability to obtain consent.
8. Conditions that will prohibit MRI scanning (metal in eye, claustrophobia, inability to lie supine).
9. Medical or psychological conditions which, in the opinion of the investigator, might create undue risk to the subject or interfere with the subject's ability to comply with the protocol requirements

Where this trial is running

Durham, North Carolina

• Duke University Medical Center — Durham, North Carolina, United States (RECRUITING)

Study contacts

• Principal investigator: Sudarshan Rajagopal, MD, PhD — Duke University
• Study coordinator: Claudia Salazar

Conditions: Interstitial Lung Disease, COPD, Pulmonary Arterial Hypertension, Lung Transplant, Group 3 PH