Chest
Original ResearchPulmonary Vascular DiseaseThe Changing Picture of Patients With Pulmonary Arterial Hypertension in the United States: How REVEAL Differs From Historic and Non-US Contemporary Registries
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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Funding/Support: This study was funded by Actelion Pharmaceuticals US, Inc.