Chest
Original ResearchPulmonary Vascular DiseaseEvaluation of the Predictive Value of a Clinical Worsening Definition Using 2-Year Outcomes in Patients With Pulmonary Arterial Hypertension: A REVEAL Registry Analysis
Section snippets
Study Population
The design and baseline characteristics of patients enrolled in the REVEAL Registry have been previously described.7, 8 The REVEAL Registry is a large, multicenter, observational, US-based study that provides current information about demographics, disease progression, and management of patients with confirmed group 1 pulmonary hypertension (ie, PAH) and represents a cohort of patients similar to those seen in routine clinical practice. Patients are followed for a minimum of 5 years from the
Patient Characteristics
At the data collection end point on September 15, 2011, 3,001 REVEAL Registry patients met the analysis criteria of age > 18 years at the time of diagnosis and PCWP ≤ 15 mm Hg (Fig 1). Of those patients, 839 were newly diagnosed (diagnostic RHC < 3 months before enrollment). Patient characteristics at baseline and in the first year of follow-up are summarized in Table 1. Age and sex were similar between patients who clinically worsened in the first year of follow-up (n = 1,340) and patients who
Discussion
Several registries and open-label, long-term extension studies of prior placebo-controlled randomized clinical trials have assessed both survival and predictors of survival. These published data demonstrate that overall survival in the contemporary treatment era is improved compared with survival data from the original National Institutes of Health registry.11
One-year survival in patients with PAH (idiopathic PAH and associated PAH) varies from as high as 96%12 to 79%.13 Contemporary 1-year
Acknowledgments
Author contributions: Dr Frost had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Dr Frost: contributed to the study design; data collection, analysis, and interpretation; and drafting, critical review, and final approval of the manuscript.
Dr Badesch: contributed to the study design; data collection, analysis, and interpretation; and drafting, critical review, and final approval of the manuscript.
Mr Miller:
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Funding/Support: Preparation of this manuscript was supported by Actelion Pharmaceuticals US, Inc. Funding and support for the REVEAL Registry was provided by CoTherix, Inc, and its affiliate Actelion Pharmaceuticals US, Inc.
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