Patients with PAH (Group 1) will be recruited to the National Institutes of Health (NIH) and enrolled in this natural history study investigating the ability of circulating markers of vascular inflammation as well as high-resolution cardiac magnetic resonance imaging (MRI) to accurately stage severity of disease and/or predict clinically relevant outcomes.
National Institutes of Health
Steven Nathan, MD
- The following parameters on RHC are required to meet the hemodynamic definition of PAH (NYHA/WHO Group I PH)
- Mean pulmonary artery pressure of greater than 25 mmHg at rest
- Pulmonary capillary wedge pressure of less than or equal to 15 mmHg (or a left ventricular end-diastolic pressure of less than or equal to 12mmHg)
- Pulmonary vascular resistance of greater than 3 Wood units (240 dyn s cm(5))
- For patients with suspected PAH (Group I PH) who have not undergone a RHC and/or additional testing to confirm the diagnosis, this testing will be completed as clinically indicated under a procedural consent. If clinically indicated (diagnostic) testing indicates that the subject with suspected PAH does not in fact meet standard criteria for PAH (Group I PH), then the subject will be removed from the study.
- Pregnant or breastfeeding women (all women of childbearing potential will be required to have a screening urine or blood pregnancy test)
- Age less than 18 years
- Inability to provide informed written consent for participation in the study