Overview

The goal of this NIH-sponsored study is to characterize three biomarkers derived from 129Xe gas exchange MRI and to understand how they change in response to interventions.

Eligibility

Inclusion Criteria for Healthy Volunteers:

        (Inclusion Criteria: Subjects must meet all of the following inclusion criteria to be
        eligible for enrollment into the trial)
          1. Outpatients of either gender, age > 18
          2. Willing and able to give informed consent and adhere to visit/protocol (Consent must
             be given before any study procedures are performed.)
          3. Subject has no diagnosed pulmonary conditions
          4. Subject has not smoked in the previous 5 years
          5. Smoking history, if any, is less than or equal to 5 pack-years
          6. No history of using other inhaled products more than 1/week for > 1 year
        Inclusion Criteria for Transfusion and Phlebotomy Patients:
          1. In-patient or outpatients of either sex, age > 18
          2. Willing and able to give informed consent and adhere to visit/protocol
        And one of the following:
          1. Patients who are scheduled to receive a red cell transfusion for anemia.
          2. Patients who are scheduled to undergo therapeutic phlebotomy to treat erythrocytosis
             or polycythemia
          3. Healthy volunteers undergoing voluntary whole blood donation (healthy volunteer
             inclusion criteria noted above)
        Inclusion Criteria for Oxygen Administration Patients:
          1. In-patient or outpatients of either sex, age > 18
          2. Willing and able to give informed consent and adhere to visit/protocol
        And one of the following categories (ILD, CTEPH, or Healthy):
          1. Interstitial Lung Disease
               -  Physician diagnosis of Interstitial Lung Disease by a pulmonologist using
                  established criteria
             OR
          2. Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
               -  Patients with a diagnosis of CTEPH, defined as mean PA pressure >20 mmHg with a
                  pulmonary vascular resistance >2 WU and pulmonary capillary wedge pressure ≤15
                  mmHg at right heart catheterization with evidence of chronic thromboembolic
                  disease on clinical imaging after 3 months of anticoagulation
               -  Scheduled for pulmonary thromboendoarterectomy (PTE) surgery and willing to
                  re-turn 3-6 months after for optional follow-up scans
             OR
          3. Healthy Volunteer (criteria noted above)
        Inclusion Criteria for Acute or Chronic Pulmonary Embolism Patients:
          1. In-patient or outpatients of either sex, age > 18
          2. Willing and able to give informed consent and adhere to visit/protocol
        And one of the following categories (Acute or Chronic)
          1. Acute Pulmonary Embolism
               -  Patients presenting with acute PE 24-48hrs post-admission
               -  Willing to return after 3-6 months of anti-coagulation therapy
             OR
          2. Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
               -  Patients with a diagnosis of CTEPH, defined as mean PA pressure >20 mmHg with a
                  pulmonary vascular resistance >2 WU and pulmonary capillary wedge pressure ≤15
                  mmHg at right heart catheterization with evidence of chronic thromboembolic
                  disease on clinical imaging after 3 months of anticoagulation
               -  Scheduled for pulmonary thromboendoarterectomy (PTE) surgery and willing to
                  re-turn 3-6 months after for optional follow-up scans
        Exclusion Criteria for All subjects:
        Subjects presenting with any of the following will not be included in the trial:
          1. MRI is contraindicated based on responses to MRI screening questionnaire
          2. Subject is pregnant or lactating
          3. Resting O2 saturation <90% with maximum supplemental O2 delivered by nasal canula
          4. Respiratory illness of a bacterial or viral etiology within 30 days of MRI
          5. Subject has history of any known ventricular cardiac arrhythmia
          6. Subject has history of cardiac arrest within the last year
          7. Subject does not fit into 129Xe vest coil used for MRI
          8. Subject cannot hold his/her breath for 10 seconds
          9. Subject deemed unlikely to be able to comply with instructions during imaging
         10. 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