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Original Research: PULMONARY VASCULAR DISEASE |

The Changing Picture of Patients With Pulmonary Arterial Hypertension in the United States: How REVEAL Differs From Historic and Non-US Contemporary Registries

Adaani E. Frost, MD, FCCP; David B. Badesch, MD; Robyn J. Barst, MD, FCCP; Raymond L. Benza, MD; C. Gregory Elliott, MD, FCCP; Harrison W. Farber, MD, FCCP; Abby Krichman, RRT; Theodore G. Liou, MD, FCCP; Gary E. Raskob, PhD; Prieya Wason, CCRP; Kathleen Feldkircher, PhD; Michelle Turner, MS; Michael D. McGoon, MD, FCCP
Author and Funding Information

From the Baylor College of Medicine (Dr Frost), Houston, TX; University of Colorado and Health Sciences Center (Dr Badesch), Denver, CO; Columbia University College of Physicians & Surgeons (Dr Barst), New York, NY; Allegheny General Hospital (Dr Benza), Pittsburgh, PA; Intermountain Medical Center, University of Utah School of Medicine (Dr Elliott), Salt Lake City, UT; Boston University School of Medicine (Dr Farber), Boston, MA; Duke University Medical Center (Ms Krichman), Durham, NC; University of Utah (Dr Liou), Salt Lake City, UT; University of Oklahoma Health Sciences Center (Dr Raskob), Oklahoma City, OK; Actelion Pharmaceuticals US, Inc (Ms Wason and Dr Feldkircher), South San Francisco, CA; ICON Clinical Research (Ms Turner), San Francisco, CA; and Mayo Clinic (Dr McGoon), Rochester, MN.

Correspondence to: Adaani E. Frost, MD, FCCP, Baylor College of Medicine, 6620 Main St, Ste 1225, Houston, TX 77030; e-mail: frost@bcm.tmc.edu


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).

Funding/Support: This study was funded by Actelion Pharmaceuticals US, Inc.


© 2011 American College of Chest Physicians


Chest. 2011;139(1):128-137. doi:10.1378/chest.10-0075
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Background:  REVEAL (The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) provides current demographics of patients with group 1 pulmonary arterial hypertension (PAH) in the United States.

Methods:  A total of 2,967 patients with PAH diagnosed based on right-sided heart catheterization were enrolled in REVEAL between March 2006 and September 2007. Demographics from the REVEAL patient cohort and REVEAL subpopulations (matched by inclusion criteria to other registries) were compared with historic US registry data and other contemporary US and non-US national PAH registries by inclusion criteria, including the National Institutes of Health (NIH) PAH registry and the French PAH registry.

Results:  REVEAL patients matched to NIH registry patients were older at diagnosis (mean ± SE, 44.9 ± 0.6 years vs 36.4 ± 1.1 years; difference, 8.5 ± 1.4; P < .001) and more likely to be women (78.7 ± 1.2% vs 63.1 ± 3.5%; P < .001). REVEAL patients matched to French registry patients had similar age and severity at diagnosis, but REVEAL patients were more likely to be women (79.8 ± 0.8% vs 65.3 ± 1.8%; P < .001) and obese (BMI, ≥ 30 kg/m2, 32.5 ± 1.0% vs 14.8 ± 1.4%; P < .001), whereas French patients were more likely to have HIV-associated PAH (6.2% vs 2.3%). The female preponderance is similar to that in other US-based contemporary registries.

Conclusions:  At diagnosis, REVEAL patients were older than NIH registry patients and similar in age to patients enrolled in contemporary registries. Compared with NIH and contemporary European and UK registries, there was a striking preponderance of women, and REVEAL patients were more likely to be obese. These observations and the difference in HIV-associated PAH between REVEAL and other non-US contemporary registries warrant further investigation.

Trial registry:  ClinicalTrials.gov; No.: NCT00370214; URL: clinicaltrials.gov.

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