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Natural History Study of Biomarkers in Pulmonary Arterial Hypertension

A Natural History Study of Novel Biomarkers in Pulmonary Arterial Hypertension

Who can join

As published by the study on ClinicalTrials.gov. "Not stated" means the study hasn't said — ask the study team.

Age
18 Years to 100 Years
Sex
All sexes
Healthy volunteers
Accepted
Compensation
Not stated

Other requirements are listed under Eligibility below. Only the study team can confirm whether you can join.

What to expect

No one can promise a trial will help you personally — that is what the research is trying to find out.

What participation involves

  • Visits mentioned: 1 visit
  • Time periods mentioned: 3 days
  • Procedures mentioned: Urine samples, Physical exams, MRI scans

Only what the study's summary and detailed description on ClinicalTrials.gov say. Time periods may describe the whole study or a part of it — ask the study team what applies to you.

Show the study's own words
  • "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"
  • "Objectives: Patients with IPAH and disease-associated PAH will be recruited to the 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."
  • "Plasma markers of endothelial inflammation, PBMC expression profiles, and high-resolution cardiac MRI will also be studied in age and sex matched controls to define normal ranges and variability for each of these novel assessments."
  • "Likewise, baseline clinical evaluations of PAH subjects will be used to examine whether any novel test (inflammatory markers, or cardiac MRI), accurately classifies patients according to their disease severity."
Compensation
Not stated
  • Study-related exams and procedures are typically provided at no cost. This is how many trials work in general — confirm the details for this study with the study team.
  • Participants are monitored closely by the research team throughout the study.
  • Some participants may receive access to an investigational treatment, which is still being studied.
  • Your participation helps researchers learn whether this approach could help future patients.

About this study

In plain language

This study follows adults with pulmonary arterial hypertension (PAH), a type of high blood pressure in the lungs, to learn how it changes over time. Researchers will compare people with PAH to healthy adults to help find targets for future treatments.

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

Compensation is read from the study's own description and may be missing — always confirm with the study team.

Eligibility

Who can join

  • You must be at least 18 years old and able to give written consent to join.
  • You may join if you have confirmed or suspected PAH. Heart pressure tests must confirm PAH for you to stay in the study.
  • Healthy volunteers must match a person with PAH in age and sex.
  • Healthy volunteers must be able to safely have MRI and CT scans, including scans with contrast dye.

Who can't join

  • Anyone: You cannot join if you are pregnant or breastfeeding.
  • Healthy volunteers: You cannot join with signs or symptoms of heart disease, more than one major heart artery disease risk factor besides age and sex, or a body mass index (BMI) over 30.
  • Healthy volunteers: Conditions linked to high lung blood pressure rule you out. These include certain immune diseases, HIV, appetite suppressant use, long-term liver disease, long-term blood clots, heart defects from birth, low oxygen, or serious lung disease.
  • Healthy volunteers: You cannot join with poorly controlled blood pressure, use of more than two blood pressure drugs, low red blood cells or platelets, blood clotting problems, or reduced kidney function.
  • Healthy volunteers: Tobacco use for over six months in the past 10 years, or any use within three months before screening or during the study, rules you out. Past recreational drug use also rules you out, except marijuana used more than three months before screening.
  • Healthy volunteers: Researchers may exclude you if another condition could affect your safety or the study results.
  • Scan limits for everyone: Some implants or metal in the body prevent MRI scans. You may not have certain scans if you cannot fit in the scanner or tolerate it. MRI also requires lying still without monitored sedation.
  • Contrast dye limits for everyone: Certain kidney problems or serious dye allergies prevent some dye scans. For iodine dye scans, a history of multiple myeloma, a blood cancer, or taking metformin within 24 hours also rules out the scan.

Simplified for readability. The official criteria below are what the study team uses.

Official eligibility criteria

* 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 \> 25 mmHg at rest, * pulmonary capillary wedge pressure of \< 15 mmHg (or a left ventricular end-diastolic pressure of \< 12 mmHg), and * pulmonary vascular resistance of \> 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. Exclusion Criteria: * 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 INCLUSION AND EXCLUSION CRITERIA FOR HEALTHY CONTROL SUBJECTS: Inclusion Criteria for Control Subjects Any healthy man or woman who is the appropriate age and sex for matching to a PAH patient * Must be eligible for MRI and Gadolinium Based MRI studies * Must be eligible for CT and Iodine Based Contrast CT studies Exclusion Criteria for Healthy Control Subjects * Current pregnancy or breastfeeding (All women of childbearing potential will be required to have a screening urine or blood pregnancy test) * Electrocardiographic evidence of clinically relevant heart disease * Symptoms of coronary or cardiac insufficiency * More than one major risk factor for coronary artery disease (excluding age and sex) * Obesity (defined as a body mass index \> 30 kg/m\^2) * History of underlying conditions/risk factors associated with pulmonary hypertension such as collagen vascular disease, HIV infection, use of appetite suppressants, chronic liver disease or cirrhosis of the liver, chronic thromboembolic disease, congenital heart defects, hypoxemia and/or significant pulmonary parenchymal disease * Systemic hypertension that is not well controlled (i.e. blood pressure at the time of screening \>140/90 mmHg in adults \< 60 years old or \>150/90 mmHg in adults 60 years or older) on medications. Subjects taking \> 2 anti-hypertensive medications will be excluded irrespective of their current blood pressure at time of screening * Anemia, thrombocytopenia or coagulopathy * Renal insufficiency (defined as an estimated glomerular filtration rate of \< 60 mL/min/1.73m\^2 of body surface area) * Active tobacco use (\> 6 months) in the past ten years, any tobacco use within 3 months prior to the screening evaluation or any tobacco use prior to completion of the study * Inability to provide informed written consent for participation in the study * History of recreational drug use with the exception of marijuana (as long as marijuana use was \> 3 months from the time of study screening). * Volunteers may be excluded if in the opinion of the study investigators they have a condition that may adversely affect the outcome of the study or the safety of the volunteer. Exclusion Criteria for MRI in Healthy Control Subjects and Subjects with PAH These contraindications include but are not limited to the following devices or conditions: 1. Implanted cardiac pacemaker or defibrillator 2. Cochlear Implants 3. Ocular foreign body (e.g. metal shavings) 4. Embedded shrapnel fragments 5. Central nervous system aneurysm clips 6. Implanted neural stimulator 7. Any implanted device that is incompatible with MRI 8. Unsatisfactory performance status as judged by the referring physician such that the subject could not tolerate an MRI scan. Examples of medical conditions that would not be accepted would include unstable angina and severe dyspnea at rest 9. Subjects requiring monitored sedation for MRI studies 10. Subjects with a condition precluding entry into the scanner (e.g. morbid obesity, claustrophobia, etc.) 11. Subjects with severe back-pain or motion disorders who will be unable to tolerate supine positioning within the MRI scanner and hold still for the duration of the examination. Exclusion Criteria for Gadolinium Based MRI Studies Only: 1. History of severe allergic reaction to gadolinium contrast agents despite pre- medication with diphenhydramine and prednisone 2. Chronic kidney disease (an estimated glomerular filtration rate of \< 60 mL/min/1.73m\^2 of body surface area) Exclusion Criteria for Cardiac Computed Tomography in Healthy Control Subjects and Subjects with PAH: 1\) Subjects with a condition precluding entry into the scanner (e.g. morbid obesity, claustrophobia, etc.) Exclusion Criteria for Iodine Based Contrast CTA Studies Only: 1. Serum creatinine \> 1.4 mg/dL 2. History of multiple myeloma 3. Use of metformin-containing products less than 24 hours prior to contrast administration 4. History of significant allergic reaction to CTA contrast agents despite pre-medication with diphenhydramine and prednisone

Study sites (1)

MD