REVEAL Registry™: Registry to Evaluate Early And Long-term PAH Disease Management

NCT ID: NCT00370214

Last Updated: 2013-05-17

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

3515 participants

Study Classification

OBSERVATIONAL

Study Start Date

2006-03-31

Study Completion Date

2012-12-31

Brief Summary

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The REVEAL Registry™ is a multicenter, observational, U.S.-based study of the clinical course and disease management of pulmonary arterial hypertension (PAH) patients. All consecutive consenting patients diagnosed with WHO Group I PAH according to specific hemodynamic criteria at participating institutions will be enrolled. Participating patients will be followed for a minimum of five years from the time of enrollment.

Detailed Description

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Pulmonary arterial hypertension (PAH) is a chronic, progressive, and often fatal disease characterized by severe constriction of the blood vessels in the lungs. Over the past decade, significant strides have been made in the medical management and understanding of PAH. However, much remains to be learned about the clinical course of both idiopathic PAH and associated PAH, including clinical presentation, pace of progression, key parameters to monitor, impact of treatment, and prognosticators of outcome. As the unique body of knowledge generated by the REVEAL Registry grows, it is hoped that new understandings, insights, and treatments will emerge that will improve the lives of patients with PAH.

The REVEAL Registry will provide investigators with descriptive data regarding the clinical course and treatment outcomes in patients with WHO Group I PAH. Data derived from the study may offer important tools for assessing current management practices of treating investigators, as well as changes over time. Additionally, the relationship of patient- and disease-specific parameters to patient outcomes may be able to be assessed through analysis of data from this study.

The specific objectives of the REVEAL Registry™ are to:

* Characterize the demographics and clinical course of PAH patients
* Evaluate and compare patient outcomes
* Identify clinical predictors of short-term and long-term clinical outcomes
* Assess the relationship between PAH medications and patient outcomes
* Report temporal trends in treatments and outcomes for newly diagnosed patients
* Collect timely and relevant data for the evolving research needs of the PAH community

Conditions

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Pulmonary Hypertension

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Eligibility Criteria

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Inclusion Criteria

* Newly diagnosed or previously diagnosed patients with WHO Group I PAH.
* Documentation of the following hemodynamic parameters by right heart catheterization, performed at any time prior to study enrollment:
* Mean pulmonary arterial pressure (mPAP) \>25 mm Hg at rest or mPAP \> 30 mm Hg with exercise contemporaneous with a pulmonary wedge pressure ≤ 18 mm Hg
* Pulmonary wedge pressure ≤ 18 mm Hg
* Pulmonary vascular resistance (PVR) ≥ 240 dynes.sec.cm-5 (i.e., ≥ 3.0 Wood units)

Exclusion Criteria

* Patients who meet the criteria for inclusion into WHO Groups II, III, IV or V
* Have not had documentation of hemodynamic criteria for PAH by right heart catheterization at some time preceding study entry and following development of symptoms associated with PAH.
* Do not meet the required hemodynamic criteria for entry into the study
Minimum Eligible Age

3 Months

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Actelion

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Michael D. McGoon, M.D.

Role: STUDY_CHAIR

Mayo Clinic - Rochester, Minnesota

David B. Badesch, M.D.

Role: STUDY_DIRECTOR

University of Colorado, Denver

Robyn J. Barst, M.D.

Role: STUDY_DIRECTOR

Columbia University

Raymond Benza, M.D.

Role: STUDY_DIRECTOR

University of Alabama at Birmingham

Gregory Elliott, M.D.

Role: STUDY_DIRECTOR

LDS Hospital

Harrison Farber, M.D.

Role: STUDY_DIRECTOR

Boston Medical Center

Adaani Frost, M.D.

Role: STUDY_DIRECTOR

Baylor College of Medicine

Abby Krichman, RRT

Role: STUDY_DIRECTOR

Duke University Pulmonary Vascular Disease Center

Locations

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University of Alabama at Birmingham

Birmingham, Alabama, United States

Site Status

University of Arizona Medical Center

Tucson, Arizona, United States

Site Status

University of California, San Diego

La Jolla, California, United States

Site Status

UCLA / VA Medical Center

Los Angeles, California, United States

Site Status

UCLA Medical Center

Los Angeles, California, United States

Site Status

Univeristy of California, San Francisco - Division of Cardiology and Pediatric ICU

San Francisco, California, United States

Site Status

Stanford University Medical Center

Stanford, California, United States

Site Status

UCLA School of Medicine: Harbor-UCLA

Torrance, California, United States

Site Status

The Children's Hospital

Denver, Colorado, United States

Site Status

University of Colorado Health Sciences Center

Denver, Colorado, United States

Site Status

University of Florida

Gainesville, Florida, United States

Site Status

Mayo Clinic

Jacksonville, Florida, United States

Site Status

Suncoast Lung Center

Sarasota, Florida, United States

Site Status

Medical College of Georgia

Augusta, Georgia, United States

Site Status

University of Chicago Hospitals

Chicago, Illinois, United States

Site Status

Midwest Heart Foundation

Naperville, Illinois, United States

Site Status

University of Iowa Health Care

Iowa City, Iowa, United States

Site Status

Kentucky Pulmonary Associates

Louisville, Kentucky, United States

Site Status

University of Maryland

Baltimore, Maryland, United States

Site Status

Johns Hopkins

Baltimore, Maryland, United States

Site Status

Tufts-New England Medical Center

Boston, Massachusetts, United States

Site Status

Massachusetts General Hospital

Boston, Massachusetts, United States

Site Status

Boston University School of Medicine

Boston, Massachusetts, United States

Site Status

Wayne State University

Detroit, Michigan, United States

Site Status

Spectrum Health Hospitals

Grand Rapids, Michigan, United States

Site Status

University of Minnesota Medical Center

Minneapolis, Minnesota, United States

Site Status

Mayo Clinic

Rochester, Minnesota, United States

Site Status

Washington University School of Medicine

St Louis, Missouri, United States

Site Status

North Shore University Hospital

Manhasset, New York, United States

Site Status

Beth Israel Medical Center

New York, New York, United States

Site Status

Columbia University

New York, New York, United States

Site Status

University of Rochester Medical Center

Rochester, New York, United States

Site Status

Duke University Medical Center

Durham, North Carolina, United States

Site Status

Cleveland Clinic Foundation

Cleveland, Ohio, United States

Site Status

University Hospital of Cleveland

Cleveland, Ohio, United States

Site Status

Children's Hospital

Columbus, Ohio, United States

Site Status

Ohio State University

Columbus, Ohio, United States

Site Status

Legacy Pulmonary Hypertension Clinic

Portland, Oregon, United States

Site Status

Penn Presbyterian Medial Center

Philadelphia, Pennsylvania, United States

Site Status

Temple Lung Center

Philadelphia, Pennsylvania, United States

Site Status

Allegheny General Hospital

Pittsburgh, Pennsylvania, United States

Site Status

University of Pittsburgh Medical Center

Pittsburgh, Pennsylvania, United States

Site Status

Rhode Island Hospital

Providence, Rhode Island, United States

Site Status

Medical University of South Carolina

Charleston, South Carolina, United States

Site Status

Vanderbilt Children's Hospital

Nashville, Tennessee, United States

Site Status

Vanderbilt University Medical Center

Nashville, Tennessee, United States

Site Status

Baylor College of Medicine

Houston, Texas, United States

Site Status

Texas Children's Hospital

Houston, Texas, United States

Site Status

University Texas Health Science Center

San Antonio, Texas, United States

Site Status

Intermountain Medical Center

Murray, Utah, United States

Site Status

Inova Heart and Vascular Institute

Falls Church, Virginia, United States

Site Status

Virginia Commonwealth University

Richmond, Virginia, United States

Site Status

Seattle Children's

Seattle, Washington, United States

Site Status

Aurora St. Luke's Medical Center

Milwaukee, Wisconsin, United States

Site Status

Countries

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United States

References

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Benza RL, Kanwar MK, Raina A, Scott JV, Zhao CL, Selej M, Elliott CG, Farber HW. Development and Validation of an Abridged Version of the REVEAL 2.0 Risk Score Calculator, REVEAL Lite 2, for Use in Patients With Pulmonary Arterial Hypertension. Chest. 2021 Jan;159(1):337-346. doi: 10.1016/j.chest.2020.08.2069. Epub 2020 Sep 1.

Reference Type DERIVED
PMID: 32882243 (View on PubMed)

Torbicki A, Bacchi M, Delcroix M, Farber HW, Ghofrani HA, Hennessy B, Jansa P, Mehta S, Perchenet L, Pulido T, Rosenberg D, Rubin LJ, Sastry BKS, Simonneau G, Sitbon O, Souza R, Wei LJ, Channick R, Benza R. Integrating Data From Randomized Controlled Trials and Observational Studies to Assess Survival in Rare Diseases. Circ Cardiovasc Qual Outcomes. 2019 May;12(5):e005095. doi: 10.1161/CIRCOUTCOMES.118.005095.

Reference Type DERIVED
PMID: 31109190 (View on PubMed)

Benza RL, Gomberg-Maitland M, Elliott CG, Farber HW, Foreman AJ, Frost AE, McGoon MD, Pasta DJ, Selej M, Burger CD, Frantz RP. Predicting Survival in Patients With Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies. Chest. 2019 Aug;156(2):323-337. doi: 10.1016/j.chest.2019.02.004. Epub 2019 Feb 14.

Reference Type DERIVED
PMID: 30772387 (View on PubMed)

Preston IR, Roberts KE, Miller DP, Sen GP, Selej M, Benton WW, Hill NS, Farber HW. Effect of Warfarin Treatment on Survival of Patients With Pulmonary Arterial Hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL). Circulation. 2015 Dec 22;132(25):2403-11. doi: 10.1161/CIRCULATIONAHA.115.018435. Epub 2015 Oct 28.

Reference Type DERIVED
PMID: 26510696 (View on PubMed)

Farber HW, Miller DP, Poms AD, Badesch DB, Frost AE, Muros-Le Rouzic E, Romero AJ, Benton WW, Elliott CG, McGoon MD, Benza RL. Five-Year outcomes of patients enrolled in the REVEAL Registry. Chest. 2015 Oct;148(4):1043-54. doi: 10.1378/chest.15-0300.

Reference Type DERIVED
PMID: 26066077 (View on PubMed)

Chung L, Farber HW, Benza R, Miller DP, Parsons L, Hassoun PM, McGoon M, Nicolls MR, Zamanian RT. Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry. Chest. 2014 Dec;146(6):1494-1504. doi: 10.1378/chest.13-3014.

Reference Type DERIVED
PMID: 24992469 (View on PubMed)

Burger CD, Long PK, Shah MR, McGoon MD, Miller DP, Romero AJ, Benton WW, Safford RE. Characterization of first-time hospitalizations in patients with newly diagnosed pulmonary arterial hypertension in the REVEAL registry. Chest. 2014 Nov;146(5):1263-1273. doi: 10.1378/chest.14-0193.

Reference Type DERIVED
PMID: 24901386 (View on PubMed)

Frost AE, Badesch DB, Miller DP, Benza RL, Meltzer LA, McGoon MD. Evaluation of the predictive value of a clinical worsening definition using 2-year outcomes in patients with pulmonary arterial hypertension: a REVEAL Registry analysis. Chest. 2013 Nov;144(5):1521-1529. doi: 10.1378/chest.12-3023.

Reference Type DERIVED
PMID: 23907471 (View on PubMed)

Bersohn MM, Turner MP, Traiger GL, Frost AE, Shapiro S. Systemic BP and heart rate as prognostic indicators in pulmonary arterial hypertension. Chest. 2013 Sep;144(3):959-965. doi: 10.1378/chest.12-2572.

Reference Type DERIVED
PMID: 23598723 (View on PubMed)

Barst RJ, Chung L, Zamanian RT, Turner M, McGoon MD. Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest. 2013 Jul;144(1):160-168. doi: 10.1378/chest.12-2417.

Reference Type DERIVED
PMID: 23429998 (View on PubMed)

Poms AD, Turner M, Farber HW, Meltzer LA, McGoon MD. Comorbid conditions and outcomes in patients with pulmonary arterial hypertension: a REVEAL registry analysis. Chest. 2013 Jul;144(1):169-176. doi: 10.1378/chest.11-3241.

Reference Type DERIVED
PMID: 23348820 (View on PubMed)

Kitterman N, Poms A, Miller DP, Lombardi S, Farber HW, Barst RJ. Bloodstream infections in patients with pulmonary arterial hypertension treated with intravenous prostanoids: insights from the REVEAL REGISTRY(R). Mayo Clin Proc. 2012 Sep;87(9):825-34. doi: 10.1016/j.mayocp.2012.05.014. Epub 2012 Aug 9.

Reference Type DERIVED
PMID: 22883740 (View on PubMed)

Frost AE, Farber HW, Barst RJ, Miller DP, Elliott CG, McGoon MD. Demographics and outcomes of patients diagnosed with pulmonary hypertension with pulmonary capillary wedge pressures 16 to 18 mm Hg: insights from the REVEAL Registry. Chest. 2013 Jan;143(1):185-195. doi: 10.1378/chest.11-1387.

Reference Type DERIVED
PMID: 22661451 (View on PubMed)

Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012 Aug;142(2):448-456. doi: 10.1378/chest.11-1460.

Reference Type DERIVED
PMID: 22281797 (View on PubMed)

Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation. 2012 Jan 3;125(1):113-22. doi: 10.1161/CIRCULATIONAHA.111.026591. Epub 2011 Nov 15.

Reference Type DERIVED
PMID: 22086881 (View on PubMed)

Krowka MJ, Miller DP, Barst RJ, Taichman D, Dweik RA, Badesch DB, McGoon MD. Portopulmonary hypertension: a report from the US-based REVEAL Registry. Chest. 2012 Apr;141(4):906-915. doi: 10.1378/chest.11-0160. Epub 2011 Jul 21.

Reference Type DERIVED
PMID: 21778257 (View on PubMed)

Shapiro S, Traiger GL, Turner M, McGoon MD, Wason P, Barst RJ. Sex differences in the diagnosis, treatment, and outcome of patients with pulmonary arterial hypertension enrolled in the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Chest. 2012 Feb;141(2):363-373. doi: 10.1378/chest.10-3114. Epub 2011 Jul 14.

Reference Type DERIVED
PMID: 21757572 (View on PubMed)

Benza RL, Gomberg-Maitland M, Miller DP, Frost A, Frantz RP, Foreman AJ, Badesch DB, McGoon MD. The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012 Feb;141(2):354-362. doi: 10.1378/chest.11-0676. Epub 2011 Jun 16.

Reference Type DERIVED
PMID: 21680644 (View on PubMed)

Brown LM, Chen H, Halpern S, Taichman D, McGoon MD, Farber HW, Frost AE, Liou TG, Turner M, Feldkircher K, Miller DP, Elliott CG. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. Chest. 2011 Jul;140(1):19-26. doi: 10.1378/chest.10-1166. Epub 2011 Mar 10.

Reference Type DERIVED
PMID: 21393391 (View on PubMed)

Burger CD, Foreman AJ, Miller DP, Safford RE, McGoon MD, Badesch DB. Comparison of body habitus in patients with pulmonary arterial hypertension enrolled in the Registry to Evaluate Early and Long-term PAH Disease Management with normative values from the National Health and Nutrition Examination Survey. Mayo Clin Proc. 2011 Feb;86(2):105-12. doi: 10.4065/mcp.2010.0394.

Reference Type DERIVED
PMID: 21282484 (View on PubMed)

Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS, Frost A, Barst RJ, Badesch DB, Elliott CG, Liou TG, McGoon MD. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010 Jul 13;122(2):164-72. doi: 10.1161/CIRCULATIONAHA.109.898122. Epub 2010 Jun 28.

Reference Type DERIVED
PMID: 20585012 (View on PubMed)

Frost AE, Badesch DB, Barst RJ, Benza RL, Elliott CG, Farber HW, Krichman A, Liou TG, Raskob GE, Wason P, Feldkircher K, Turner M, McGoon MD. The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US Contemporary Registries. Chest. 2011 Jan;139(1):128-37. doi: 10.1378/chest.10-0075. Epub 2010 Jun 17.

Reference Type DERIVED
PMID: 20558556 (View on PubMed)

Chung L, Liu J, Parsons L, Hassoun PM, McGoon M, Badesch DB, Miller DP, Nicolls MR, Zamanian RT. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010 Dec;138(6):1383-94. doi: 10.1378/chest.10-0260. Epub 2010 May 27.

Reference Type DERIVED
PMID: 20507945 (View on PubMed)

Related Links

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http://www.REVEALregistry.com

Official REVEAL Registry website

Other Identifiers

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CR001

Identifier Type: -

Identifier Source: org_study_id

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