Chest
Volume 142, Issue 2, August 2012, Pages 448-456
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Original Research
Pulmonary Vascular Disease
An Evaluation of Long-term Survival From Time of Diagnosis in Pulmonary Arterial Hypertension From the REVEAL Registry

https://doi.org/10.1378/chest.11-1460Get rights and content

Background

The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry) was established to characterize the clinical course, treatment, and predictors of outcomes in patients with pulmonary arterial hypertension (PAH) in the United States. To date, estimated survival based on time of patient enrollment has been established and reported. To determine whether the survival of patients with PAH has improved over recent decades, we assessed survival from time of diagnosis for the REVEAL Registry cohort and compared these results to the estimated survival using the National Institutes of Health (NIH) prognostic equation.

Methods

Newly or previously diagnosed patients (aged ≥ 3 months at diagnosis) with PAH enrolled from March 2006 to December 2009 at 55 US centers were included in the current analysis.

Results

A total of 2,635 patients qualified for this analysis. One-, 3-, 5-, and 7-year survival rates from time of diagnostic right-sided heart catheterization were 85%, 68%, 57%, and 49%, respectively. For patients with idiopathic/familial PAH, survival rates were 91% ± 2%, 74% ± 2%, 65% ± 3%, and 59% ± 3% compared with estimated survival rates of 68%, 47%, 36%, and 32%, respectively, using the NIH equation.

Conclusions

Comprehensive analysis of survival from time of diagnosis in a large cohort of patients with PAH suggests considerable improvements in survival in the past 2 decades since the establishment of the NIH registry, the effects of which most likely reflect a combination of changes in treatments, improved patient support strategies, and possibly a PAH population at variance with other cohorts.

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.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.

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