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

NCT00370214 | Completed DMC

• 1 other identifier: CR001
• Study Type: observational
• Target: 3,515
• Locations: 1 country
• Sites: 54

Brief Summary

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.

Conditions

Pulmonary Hypertension

Keywords

Pulmonary arterial hypertension; Lung diseases; Heart diseases; Pulmonary circulation; Vascular resistance; PAH; Hypertension

Outcome Measures

Primary Outcomes (1)

• Survival

  5 years

Secondary Outcomes (1)

• clinical predictors, precise outcome definitions

  5 years

Eligibility Criteria

• Age: 3 Months+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Patients with WHO Group I Pulmonary Arterial Hypertension from US based PAH centers.

You may qualify if:

• 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)

You may not qualify if:

• 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

Sponsors & Collaborators

• Actelion: lead

Study Sites (54)

University of Alabama at Birmingham

Birmingham, Alabama, 35294, United States

Location

University of Arizona Medical Center

Tucson, Arizona, 85724, United States

Location

University of California, San Diego

La Jolla, California, 92037, United States

Location

UCLA / VA Medical Center

Los Angeles, California, 90073, United States

Location

UCLA Medical Center

Los Angeles, California, 90095, United States

Location

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

San Francisco, California, 94143, United States

Location

Stanford University Medical Center

Stanford, California, 94305, United States

Location

UCLA School of Medicine: Harbor-UCLA

Torrance, California, 90502, United States

Location

The Children's Hospital

Denver, Colorado, 80218, United States

Location

University of Colorado Health Sciences Center

Denver, Colorado, 80262, United States

Location

University of Florida

Gainesville, Florida, 32610, United States

Location

Mayo Clinic

Jacksonville, Florida, 32224, United States

Location

Suncoast Lung Center

Sarasota, Florida, 34233, United States

Location

Medical College of Georgia

Augusta, Georgia, 30912, United States

Location

University of Chicago Hospitals

Chicago, Illinois, 60637, United States

Location

Midwest Heart Foundation

Naperville, Illinois, 60566, United States

Location

University of Iowa Health Care

Iowa City, Iowa, 52242, United States

Location

Kentucky Pulmonary Associates

Louisville, Kentucky, 40202, United States

Location

University of Maryland

Baltimore, Maryland, 21201, United States

Location

Johns Hopkins

Baltimore, Maryland, 21205, United States

Location

Tufts-New England Medical Center

Boston, Massachusetts, 02111, United States

Location

Massachusetts General Hospital

Boston, Massachusetts, 02114, United States

Location

Boston University School of Medicine

Boston, Massachusetts, 02118, United States

Location

Wayne State University

Detroit, Michigan, 48201, United States

Location

Spectrum Health Hospitals

Grand Rapids, Michigan, 49506, United States

Location

University of Minnesota Medical Center

Minneapolis, Minnesota, 55455, United States

Location

Mayo Clinic

Rochester, Minnesota, 55095, United States

Location

Washington University School of Medicine

St Louis, Missouri, 63021, United States

Location

North Shore University Hospital

Manhasset, New York, 11030, United States

Location

Beth Israel Medical Center

New York, New York, 10003, United States

Location

Columbia University

New York, New York, 10032, United States

Location

University of Rochester Medical Center

Rochester, New York, 14642, United States

Location

Duke University Medical Center

Durham, North Carolina, 27710, United States

Location

Cleveland Clinic Foundation

Cleveland, Ohio, 44022, United States

Location

University Hospital of Cleveland

Cleveland, Ohio, 44106, United States

Location

Children's Hospital

Columbus, Ohio, 43210, United States

Location

Ohio State University

Columbus, Ohio, 43210, United States

Location

Legacy Pulmonary Hypertension Clinic

Portland, Oregon, 97210, United States

Location

Penn Presbyterian Medial Center

Philadelphia, Pennsylvania, 19104, United States

Location

Temple Lung Center

Philadelphia, Pennsylvania, 19140, United States

Location

Allegheny General Hospital

Pittsburgh, Pennsylvania, 15212, United States

Location

University of Pittsburgh Medical Center

Pittsburgh, Pennsylvania, 15213, United States

Location

Rhode Island Hospital

Providence, Rhode Island, 02903, United States

Location

Medical University of South Carolina

Charleston, South Carolina, 29425, United States

Location

Vanderbilt Children's Hospital

Nashville, Tennessee, 37212, United States

Location

Vanderbilt University Medical Center

Nashville, Tennessee, 37232, United States

Location

Baylor College of Medicine

Houston, Texas, 77030, United States

Location

Texas Children's Hospital

Houston, Texas, 77030, United States

Location

University Texas Health Science Center

San Antonio, Texas, 78229, United States

Location

Intermountain Medical Center

Murray, Utah, 84107, United States

Location

Inova Heart and Vascular Institute

Falls Church, Virginia, 22042, United States

Location

Virginia Commonwealth University

Richmond, Virginia, 23298, United States

Location

Seattle Children's

Seattle, Washington, 98105, United States

Location

Aurora St. Luke's Medical Center

Milwaukee, Wisconsin, 53215, United States

Location

Related Publications (23)

• 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.

  PMID: 32882243 DERIVED

• 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.

  PMID: 31109190 DERIVED

• 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.

  PMID: 30772387 DERIVED

• 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.

  PMID: 26510696 DERIVED

• 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.

  PMID: 26066077 DERIVED

• 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.

  PMID: 24992469 DERIVED

• 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.

  PMID: 24901386 DERIVED

• 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.

  PMID: 23907471 DERIVED

• 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.

  PMID: 23598723 DERIVED

• 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.

  PMID: 23429998 DERIVED

• 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.

  PMID: 23348820 DERIVED

• 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.

  PMID: 22883740 DERIVED

• 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.

  PMID: 22661451 DERIVED

• 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.

  PMID: 22281797 DERIVED

• 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.

  PMID: 22086881 DERIVED

• 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.

  PMID: 21778257 DERIVED

• 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.

  PMID: 21757572 DERIVED

• 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.

  PMID: 21680644 DERIVED

• 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.

  PMID: 21393391 DERIVED

• 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.

  PMID: 21282484 DERIVED

• 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.

  PMID: 20585012 DERIVED

• 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.

  PMID: 20558556 DERIVED

• 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.

  PMID: 20507945 DERIVED

Related Links

• Official REVEAL Registry website
• PAH clinical studies at Mayo Clinic

MeSH Terms

Conditions

Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Lung Diseases; Heart Diseases; Hypertension

Condition Hierarchy (Ancestors)

Respiratory Tract Diseases; Vascular Diseases; Cardiovascular Diseases

Study Officials

• Michael D. McGoon, M.D.

  Mayo Clinic - Rochester, Minnesota

  STUDY CHAIR

• David B. Badesch, M.D.

  University of Colorado, Denver

  STUDY DIRECTOR

• Robyn J. Barst, M.D.

  Columbia University

  STUDY DIRECTOR

• Raymond Benza, M.D.

  University of Alabama at Birmingham

  STUDY DIRECTOR

• Gregory Elliott, M.D.

  LDS Hospital

  STUDY DIRECTOR

• Harrison Farber, M.D.

  Boston Medical Center

  STUDY DIRECTOR

• Adaani Frost, M.D.

  Baylor College of Medicine

  STUDY DIRECTOR

• Abby Krichman, RRT

  Duke University Pulmonary Vascular Disease Center

  STUDY DIRECTOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: INDUSTRY
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: August 29, 2006
• First Posted: August 31, 2006
• Study Start: March 1, 2006
• Primary Completion: December 1, 2012
• Study Completion: December 1, 2012
• Last Updated: May 17, 2013

Record last verified: 2013-05

Locations

US (54)