Non-Invasive Diagnosis of Pulmonary Vascular Disease Using Inhaled 129Xe Magnetic Resonance Imaging
Condition: Pulmonary Vascular Disease
MeSH terms: Vascular Diseases
Intervention: GE-141, Hyperpolarized 129Xenon gas; MRI
Intervention description: XeMRI scans will provide 3D images of ventilation and gas exchange. Subjects will inhale HP 129Xe from the dose delivery bags. Then the subject will be moved into the scanner and they will undergo basic 1H localizer and anatomical scans. Once localization is complete, subjects will under-go several MRI scans after inhalation of HPXe; Perform MRI scans with administration of GE-141, Hyperpolarized 129Xenon
Intervention MeSH terms: Xenon
Phase: Phase 2
Study type: Interventional
Primary purpose: Diagnostic
Allocation: Non-Randomized
Intervention model: Sequential Assignment
Intervention model description: This will be a single blinded open label study enrolling volunteers and patients with pure PAH (10 subjects), pure left heart disease (10 subjects) and pure lung disease (10 subjects), 5 CTEPH in Aim 1; followed by a larger cohort of 92 subjects with PAH or other cardiac or pulmonary disease for MRI scans
Masking: Single (Outcomes Assessor)
Masking description: The outcomes assessor, the radiologist who is reading the imaging will be blinded to treatment.
Enrollment: 62.0
Sex: All
Minimum age: 18 Years
Healthy volunteers: No
Keywords: right heart catheterization for evaluation of PAH or other cardiac or pulmonary disease

Summary:

      This study seeks to deploy several forms of 129Xe MRI contrast as well as emerging
      conventional proton MRI technqiues for imaging lung structure and perfusion. Specifically,
      the 129Xe MRI scans will provide 3D images of ventilation and gas exchange, and spectroscopic
      indices will be evaluated to test gas exchange dynamics with high temporal resolution. The
      conventional 1H MRI scans will include a free-breathing ultra-short echo time (UTE) scan that
      provides images similar to that of a CT scan. In addition, to characterize perfusion and
      vascular dimensions directly, patients will undergo a gadolinium-enhanced perfusion scan.
    

Eligibility criteria:

        Inclusion criteria

          1. Outpatients of either gender, age > 18

          2. Willing and able to give informed consent and adhere to visit/protocol schedules.
             (Consent must be given before any study procedures are performed)

          3. Women of childbearing potential must have a negative urine pregnancy test. This will
             be confirmed before participation in this investigational protocol.

          4. Either has a diagnosis of PAH, isolated left heart disease or lung disease (chronic
             obstructive pulmonary disease or interstitial lung disease) or CTEPH using established
             clinical criteria.

          5. Patients undergoing right heart catheterization for evaluation of PAH or other cardiac
             or pulmonary disease for MRI scans

        Exclusion criteria

          -  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

          -  Conditions that will prohibit MRI scanning (metal in eye, claustrophobia, inability to
             lie supine)