Chest

Chest

Volume 139, Issue 1, January 2011, Pages 128-137
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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

https://doi.org/10.1378/chest.10-0075Get rights and content

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.

Section snippets

Materials and Methods

For REVEAL, investigators consecutively enrolled consenting patients with group 1 PAH at 54 US sites. The registry protocol was approved by the institutional review board of each study center. All patients provided written informed consent. To better reflect patterns of practice in the United States and to be more inclusive in analysis of treatment and outcomes over time, pediatric patients (≤ 18 years of age) and patients fitting a broader hemodynamic definition of PAH than usual (PCWP ≤ 18 mm

Patient Cohort and Cohorts Matched to Prior Registries

Between March 2006 and September 2007, 2,967 patients meeting study entry criteria at rest were enrolled in REVEAL. For purposes of comparison with other PAH registries, patients in REVEAL who met the definition of PAH with exercise (mPAP > 30 mm Hg) were excluded from these analyses (n = 12), leaving 2,955 patients in the total REVEAL analysis cohort. The mean ± SE duration from diagnosis to enrollment was 38.6 ± 0.8 months (median, 26.0 months). REVEAL consisted of approximately equal numbers

Discussion

The comparison of REVEAL with the 1980s NIH registry shows several striking differences, regardless of whether only patients with IPAH or FPAH or the entire group 1 PAH patient cohort are examined. In particular, contemporary patients with PAH are older at diagnosis and overwhelmingly women. The older age at diagnosis in contemporary registries is of particular interest because patients have similar New York Heart Association and World Health Organization FCs at diagnosis as in the NIH

Acknowledgments

Author contributions: Dr Frost had access to and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Dr Frost: contributed to the study design; collection, analysis, and interpretation of data; and drafting, critical review, and final approval of the manuscript.

Dr Badesch: contributed to the study design; collection, analysis, and interpretation of data; and drafting, critical review, and final approval of the manuscript.

Dr Barst: contributed to the study

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    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.

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    Copyright © 2011 The American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.