Natural History Study of Biomarkers in Pulmonary Arterial Hypertension

NCT01730092 | Recruiting

• 2 other identifiers: 13001213-CC-0012
• Study Type: observational
• Target: 270
• Locations: 1 country
• Sites: 1

Brief 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

Conditions

Pulmonary Disease; Pulmonary Hypertension

Keywords

Vascular Inflammation; Microarray; Right Ventricular Function; Endothelial Dysfunction; Magnetic Resonance Imaging; Natural History

Outcome Measures

Primary Outcomes (1)

• Examine whether any novel test (inflammatory markers or high resolution cardiac MRI), accurately classifies PAH subjects according to disease severity as assessed by their baseline 6-minute walk distance.

  Compare outcomes in PAH subjects with controls

  10 years

Study Arms (2)

Healthy Volunteers

Enroll up to 120 healthy volunteers, in order to obtain approximately 55 evaluable healthy volunteers matched to pulmonary arterial hypertension subjects for age and sex

Pulmonary Arterial Hypertension Subjects

150 subjects with pulmonary arterial hypertension; male or female, age grater than or equal to 18 - 99 years

Eligibility Criteria

• Age: 18 Years - 100 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

primary clinical for PAH and community sample for controls

You may not qualify if:

• 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) and
• 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
• 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
• 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)

Sponsors & Collaborators

• National Institutes of Health Clinical Center (CC): lead

Study Sites (1)

National Institutes of Health Clinical Center

Bethesda, Maryland, 20892, United States

RECRUITING

Related Publications (3)

• Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, Barst RJ, Benza RL, Liou TG, Turner M, Giles S, Feldkircher K, Miller DP, McGoon MD. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010 Feb;137(2):376-87. doi: 10.1378/chest.09-1140. Epub 2009 Oct 16.

  PMID: 19837821 BACKGROUND

• Rubin LJ. Primary pulmonary hypertension. Chest. 1993 Jul;104(1):236-50. doi: 10.1378/chest.104.1.236. No abstract available.

  PMID: 8325077 BACKGROUND

• D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991 Sep 1;115(5):343-9. doi: 10.7326/0003-4819-115-5-343.

  PMID: 1863023 BACKGROUND

Related Links

• NIH Clinical Center Detailed Web Page

MeSH Terms

Conditions

Lung Diseases; Hypertension, Pulmonary

Condition Hierarchy (Ancestors)

Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Study Officials

• Michael A Solomon, M.D.

  National Institutes of Health Clinical Center (CC)

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Grace M Graninger, R.N.

CONTACT

(301) 496-9320; ggraninger@cc.nih.gov

Michael A Solomon, M.D.

CONTACT

(301) 496-9320; msolomon@cc.nih.gov

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: November 17, 2012
• First Posted: November 21, 2012
• Study Start: July 15, 2013
• Primary Completion (Estimated): December 31, 2030
• Last Updated: April 14, 2026

Record last verified: 2026-04-08

Locations

US (1)