Chest
Volume 137, Issue 2, February 2010, Pages 376-387
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Original Research
Pulmonary Hypertension
Pulmonary Arterial Hypertension: Baseline Characteristics From the REVEAL Registry

https://doi.org/10.1378/chest.09-1140Get rights and content

Background

The Registry to EValuate Early And Long-term pulmonary arterial hypertension disease management (REVEAL Registry) was established to provide updated characteristics of patients with pulmonary arterial hypertension (PAH) and to improve diagnosis, treatment, and management.

Methods

Fifty-four US centers enrolled consecutively screened patients with World Health Organization group I PAH who met expanded hemodynamic criteria of mean pulmonary arterial pressure (PAP) > 25 mm Hg at rest (30 mm Hg with exercise), pulmonary capillary wedge pressure (PCWP) ≤ 18 mm Hg, and pulmonary vascular resistance ≥ 240 dynes · s · cm−5. Patients meeting the traditional hemodynamic definition (PCWP ≤ 15 mm Hg) were compared with those with a PCWP of 16 to 18 mm Hg.

Results

Between March 2006 and September 2007, 2,967 patients enrolled. Among 2,525 adults meeting traditional hemodynamic criteria, the mean age was 53 ± 14 years, and 2,007 (79.5%) were women. The mean duration between symptom onset and diagnostic catheterization was 2.8 years, and 1,008 (41.3%) patients were treated with more than one pulmonary vascular-targeted medication. Compared with patients meeting the traditional hemodynamic definition of PAH, patients with a PCWP of 16 to 18 mm Hg were older, more obese, had a lower 6-min walk distance, and had a higher incidence of systemic hypertension, sleep apnea, renal insufficiency, and diabetes.

Conclusions

Patients in the REVEAL Registry are older and more often female than in previous descriptions. Delays between symptom onset and diagnostic catheterization persist. Many treatment regimens are fundamentally empirical, and data will be required to determine outcomes, improve risk stratification, and develop and validate more precise prognostic tools. Patients with PCWP of 16 to 18 mm Hg differ in a number of important respects from those meeting the traditional hemodynamic definition of PAH.

Section snippets

Design Overview

The REVEAL Registry was designed by an independent Steering Committee, which oversaw study execution, data analysis, and reporting of the results. The design of the registry has been described in detail.26 The registry uses a multicenter prospective cohort design involving 54 centers in the United States (including 21 sites enrolling pediatric patients). The centers include diverse university-affiliated and community hospitals. Each of the four US Census Regions was represented.

Between March

Demographics

A total of 3,052 patients with clinically suspected WHO group I PAH were screened. Of these, 2,977 patients (97.2%) were enrolled. Ten patients were subsequently determined to have had inadvertent duplication of data; the duplicate entries were discarded, thereby yielding a final total of 2,967 patients. A total of 2,864 patients met the study entry criteria based on mean PAP, PCWP, and PVR at rest. Another 83 patients met the criteria at rest, substituting left ventricular end-diastolic

Discussion

The REVEAL Registry is the largest and most comprehensive registry of WHO group I PAH reported to date. The size of the study (2,967 patients), the number of referral sites and community practices, and geographical distribution make the results generalizable to clinical practice. No center enrolled more than 10% of the population. Importantly, by using a less-restrictive PCWP criterion of ≤ 18 mm Hg (as compared with the usual criterion of ≤ 15 mm Hg), the registry intentionally included

Acknowledgments

Author contributions: Dr Badesch: contributed to the study design; collection, analysis and interpretation of data; and drafting and critical review of the manuscript; and has seen and approved the final version.

Dr Raskob: contributed to the study design; collection, analysis and interpretation of data; drafting and critical review of the manuscript; and has seen and approved the final version.

Dr Elliott: contributed to the study design; collection, analysis and interpretation of data; drafting

References (41)

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Funding/Support: The REVEAL Registry is sponsored by Actelion Pharmaceuticals. Editorial support for the preparation of this manuscript was funded by Actelion Pharmaceuticals and provided by Jennifer M. Kulak, PhD, and Carol A. Lewis, PhD, from Wolters Kluwer.

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

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