Chest
Original ResearchPulmonary Vascular DiseaseAn Evaluation of Long-term Survival From Time of Diagnosis in Pulmonary Arterial Hypertension From the REVEAL Registry
Section snippets
Study Design and Participants
The observational, prospective REVEAL Registry study was conducted at 55 sites in the United States (see e-Appendix 1 for a list of the REVEAL Registry sites and corresponding institutional review board numbers). The design and inclusion criteria for the REVEAL Registry have been described previously.10 The protocol was reviewed by the institutional review board of each participating center, and all participants or their legal guardians provided written informed consent (and assent in the cases
Patient Demographic, Hemodynamic, and Functional Characteristics
A total of 3,517 patients meeting all inclusion criteria were enrolled consecutively in the REVEAL Registry; 612 were excluded from this analysis because of diagnostic RHC before November 2001. Patient disposition and inclusion in each of the analyses are shown in Figure 1. The overall survival analysis included 2,635 patients, including 1,267 patients with IPAH/FPAH, of whom 985 met the NIH PCWP criteria (≤ 12 mm Hg), 755 of whom, unlike the NIH patients, were treated within 6 months of
Discussion
Our evaluation of survival from the time of diagnostic RHC in 2,635 patients with PAH enrolled in the REVEAL Registry confirms that although mortality in PAH remains unacceptably high, current survival rates have improved markedly since those reported by the NIH registry for patients diagnosed from 1981 to 1984.3 Furthermore, a substantial improvement in survival is observed in the subgroup of REVEAL Registry patients who initiated therapy within 6 months of diagnosis and met the hemodynamic
Conclusions
In comparison with the US-based NIH registry, which predates therapies that are specific for patients with PAH, survival analyses from time of diagnostic RHC in the REVEAL Registry demonstrate a significant improvement in outcome in the current era. A median survival of 2.8 years for patients with primary PH was documented in the NIH registry, whereas the data from the REVEAL Registry suggest that patients with PAH with a profile similar to those in the NIH registry in the United States can
Acknowledgments
Author contributions: Dr Benza serves as the guarantor of the manuscript.
Dr Benza: contributed to the study design; collection, analysis, and interpretation of the data; drafting and critical review of the manuscript; and approval of the final version.
Mr Miller: contributed to the study design; collection, analysis, and interpretation of the data; drafting and critical review of the manuscript; and approval of the final version.
Dr Barst: contributed to the study design; collection, analysis,
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Funding/Support: Preparation of this manuscript was supported by Actelion Pharmaceuticals US, Inc. Funding for the REVEAL Registry is provided by Actelion Pharmaceuticals US, Inc.
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