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Original Research: Pulmonary Vascular Disease |

Five-Year Outcomes of Patients Enrolled in the REVEAL RegistryREVEAL PAH Disease Management Five-Year Outcomes

Harrison W. Farber, MD, FCCP; Dave P. Miller, MS; Abby D. Poms, BS, RRT; David B. Badesch, MD, FCCP; Adaani E. Frost, MD; Erwan Muros-Le Rouzic, MPH; Alain J. Romero, PharmD, PhD; Wade W. Benton, PharmD; C. Gregory Elliott, MD; Michael D. McGoon, MD, FCCP; Raymond L. Benza, MD
Author and Funding Information

From the Boston University School of Medicine (Dr Farber), Boston, MA; ICON Clinical Research (Mr Miller), San Francisco, CA; Duke University Medical Center (Ms Poms), Durham, NC; University of Colorado Health Sciences Center (Dr Badesch), Denver, CO; Division of Pulmonary and Critical Care (Dr Frost), Baylor College of Medicine, Houston, TX; Actelion Pharmaceuticals Ltd (Mr Muros-Le Rouzic), Allschwil, Switzerland; Actelion Pharmaceuticals US, Inc (Drs Romero and Benton), South San Francisco, CA; Intermountain Medical Center (Dr Elliott), Murray, UT; Mayo Clinic (Dr McGoon), Rochester, MN; and Allegheny General Hospital (Dr Benza), Pittsburgh, PA.

CORRESPONDENCE TO: Harrison W. Farber, MD, FCCP, Division of Pulmonary, Critical Care, Sleep Medicine, and Allergy, The Pulmonary Center, Boston University School of Medicine, 72 E Concord St, R-304, Boston, MA 02118; e-mail: hfarber@bu.edu


Part of this article has been presented at the 2014 ATS International Conference, May 16-21, 2014, San Diego, CA.

FUNDING/SUPPORT: Actelion Pharmaceuticals US, Inc, the sponsor of the REVEAL Registry, provided funding and support for the analysis presented.

This is an open access article distributed under the terms of the Creative Commons Attribution-Noncommercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted use, distribution, and reproduction to noncommercial entities, provided the original work is properly cited. Information for reuse by commercial entities is available online.


Chest. 2015;148(4):1043-1054. doi:10.1378/chest.15-0300
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BACKGROUND:  Pulmonary arterial hypertension (PAH) is a rare, severe disease characterized by worsening right-sided heart failure, decreasing functional status, and poor survival. The present study characterizes the 5-year survival in the United States of a new and previous diagnosis of PAH in patients stratified by baseline functional class (FC). The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) is a 55-center observational US registry of the demographics, disease course, and management of patients with World Health Organization (WHO) group 1 PAH.

METHODS:  The REVEAL Registry enrolled newly and previously diagnosed patients aged ≥ 3 months with WHO group 1 PAH consecutively from March 2006 to December 2009. Demographics, disease characteristics, and hemodynamic data were collected at enrollment. Survival analysis was conducted by FC and other subgroups in patients aged ≥ 18 years.

RESULTS:  Survival differences between previously diagnosed and newly diagnosed patients at 1 year (90.4% vs 86.3%) were maintained to 5 years; 5-year survival for previously diagnosed patients was 65.4% compared with 61.2% for newly diagnosed patients. Previously diagnosed patients in FC I, II, III, and IV had an estimated 5-year survival rate of 88.0%, 75.6%, 57.0%, and 27.2%, respectively, compared with 72.2%, 71.7%, 60.0%, and 43.8% for newly diagnosed patients in FC I, II, III, and IV, respectively.

CONCLUSIONS:  Patient survival of advanced PAH remains poor at 5 years despite treatment advances. New York Heart Association FC remains one of the most important predictors of future survival. These observations reinforce the importance of continuous monitoring of FC in patients with PAH.

TRIAL REGISTRY:  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov

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