A Natural History Study of Novel Biomarkers in Pulmonary Arterial Hypertension
National Institutes of Health Clinical Center (CC)
Summary
Background: \- High blood pressure in the lungs, known as pulmonary arterial hypertension (PAH), is a rare disorder. Some people have disease-associated PAH and some have PAH from an unknown cause. Researchers want to follow the natural history of all PAH patients to understand how PAH progresses in order to discover targets for future research into new treatments. To further identify treatment targets, they will compare healthy volunteers to patients with PAH. Objectives: \- To study the natural history of PAH. Eligibility: * Individuals at least 18 years of age who have PAH. * Healthy volunteers at least 18 years of age. Design: * Participants with PAH will have periodic visits to the National Institutes of Health Clinical Center. After the first visit, they will return in 6 months and then yearly or every other year for as long as the study continues. * The first visit will take up to 3 days. It will involve the following tests: * Physical exam and medical history * Blood and urine samples * Heart and lung function tests and imaging studies * Six-minute walk test * Questions about exercise and physical activity * Healthy volunteers will have only one visit to the Clinical Center, during which they will undergo screening tests, and complete many of the same tests as patients with PAH
Description
Introduction: Pulmonary arterial hypertension (PAH) is a rare disorder associated with poor survival. Endothelial dysfunction resulting from 1) genetic susceptibility, and 2) a triggering stimulus that initiates pulmonary vascular injury, the two-hit hypothesis, appears to play a central role both in the pathogenesis and progression of PAH. Inflammation appears to drive this dysfunctional endothelial phenotype, propagating cycles of injury and repair in genetically susceptible patients with idiopathic PAH (IPAH) and patients with disease-associated PAH. However, despite mounting evidence of v…
Eligibility
- Age range
- 18–100 years
- Sex
- All
- Healthy volunteers
- Yes
* ELIGIBILITY: The study will enroll subjects with confirmed and suspected NYHA/WHO Group 1 PH (68) as well as age and sex matched control subjects. Subjects must be at least 18 years of age and must be able to provide informed, written consent for participation in this study. There is no exclusion based on race or sex. INCLUSION AND EXCLUSION CRITERIA FOR PAH SUBJECTS: Inclusion Criteria: 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 wedg…
Location
- National Institutes of Health Clinical CenterBethesda, Maryland