The overall objective outlined in this study is to determine how pulmonary vascular remodeling in PAH at a cellular and pathological level is associated with changes in gas exchange physiology and hemodynamics (monitored with 129Xe MRI/MRS) and how these signals change with disease progression or treatment.
Who can participate
Age range18 Years – 75 Years
SexALL
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Inclusion criteria
✓. Outpatients of either gender, Age 18-75
✓. Awaiting a lung transplant
✓. Diagnosis of precapillary PH (right heart catheterization demonstrating hemodynamic criteria of a mean pulmonary artery pressure (mPAP) ≥ 25 mmHg, pulmonary vascular resistance ≥ 3 WU, pulmonary capillary wedge pressure ≤ 15 mmHg) in the setting of Group 1 (PAH), 3 (PH due to chronic lung disease, 4 (PH due to pulmonary artery obstructions), or 5 (PH due to miscellaneous causes)
✓. Willing and giving informed consent and adhere to visit/protocol schedules (consent must be given before any study procedures are performed).
✓. Women of childbearing potential must have a negative urine pregnancy test before MRI
Exclusion criteria
✕. Moderate to severe heart disease (LVEF \<45%, Severe LV hypertrophy, Moderate to severe valvular disease)
✕. PH due to schistosomiasis
✕. Active cancer
✕. Sickle cell anemia
✕. Prisoners and pregnant women will not be approached for the study
✕. Conditions that will prohibit MRI scanning (metal in eye, claustrophobia, inability to lie supine)
✕. 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