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Pulmonary and Cardiovascular: Original ResearchRetrospective Validation of the REVEAL 2.0 Risk Score With the Australian and New Zealand Pulmonary Hypertension Registry Cohort
Section snippets
REVEAL Registry and REVEAL Models
The REVEAL Registry is a multicenter observational prospective registry that enrolled patients with WHO group 1 PAH at 55 US centers between 2006 and 2009.22,23 The original REVEAL risk model (N = 2,716) was developed to predict 1-year survival of incident and prevalent patients from time of registry enrollment based on 12 variables identified through multivariable Cox regression from the REVEAL cohort.7,9,14 In development of the new REVEAL 2.0 model, only patients who have survived at least 1
Study Population
Of 3,535 patients with pulmonary hypertension enrolled in the PHSANZ Registry, 2,736 adults were classified as WHO group 1 PAH and 1,632 survived at least 1 year postenrollment with follow-up data available for analyses. A further 556 patients (34%) were excluded because of absent right-sided heart catheter data or failure to meet hemodynamic definition of PAH (mean pulmonary arterial pressure ≥ 25 mm Hg and pulmonary arterial wedge pressure ≤ 15 mm Hg) at diagnosis. A total of 1,011 patients
Discussion
Multiple risk assessment tools have been proposed for prediction of mortality risk in PAH.5,9, 10, 11, 12, 13, 14 The original REVEAL risk score model was one of the first to be validated in the era of PAH-targeted therapies.9 The REVEAL 2.0 risk score modified the predictive parameters of REVEAL, and was derived from a baseline at 1 year postenrollment to allow the inclusion of all-cause hospitalization in the prior 6 months as a new parameter.24 Our study represents the first external
Conclusions
In a large external cohort, the REVEAL 2.0 risk model effectively discriminates risk in cohorts of both patients with previously diagnosed and incident PAH, supporting its use in wider populations. The three-category version of the REVEAL 2.0 risk score is particularly appealing because it conforms to the approach advocated by risk prognostication guidelines and may be easier for physicians to apply. Further work is needed to identify the optimal risk prognostication tool in PAH, and should
Acknowledgments
Author contributions: J. J. A. takes responsibility for the content of the manuscript, including the data and analysis. J. J. A., E. M. L., and G. S. conceived and designed the study. J. J. A. analyzed the data and prepared the manuscript with supervision from E. M. L. and G. S. Data were contributed by E. M. L., M. L, D. S. C., N. C., C. C., N. D., J. F., M. H., D. K., A. K., F. K., E. K., T. M., B. R., P. S., V. T., T. W., H. W., and J. P. W. All authors critically revised the manuscript for
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Cited by (23)
The evolving landscape of pulmonary arterial hypertension clinical trials
2022, The LancetCitation Excerpt :Risk scores also correlate with quality of life.110 Many risk scores have been validated retrospectively across global registries4,43,107,111 and in post-hoc analyses of clinical trials.112–114 Exploratory analyses using risk stratification have been performed in randomised control trials such as GRIPHON,15 PATENT-1,26 and AMBITION17 trials to identify subgroups of patients in whom treatment effects were more pronounced.
Does community size or commute time affect severity of illness at diagnosis or quality of care in a centralized care model of pulmonary hypertension?
2021, International Journal of CardiologyCitation Excerpt :Inhaled therapies are not available in Canada. Recent studies have shown that integrating a combination of known prognostic variables into a risk score provides superior prognostication to using single variables alone [11–13]. Clinical practice guidelines now strongly recommend basing treatment decisions on risk scores [1,14].
Blood carbon dioxide tension and risk in pulmonary arterial hypertension
2020, International Journal of CardiologyCitation Excerpt :The updated REVEAL 2.0 score was recently introduced and includes a set of 11 different parameters [15]. The prognostic value of the updated score was demonstrated in a registry study (Pulmonary Hypertension Society of Australia and New Zealand Registry) [22]. Integrating PaCO2 as an additional non-invasive criterion has the potential to improve individual risk assessment.
Risk assessment in pulmonary arterial hypertension: Insights from the GRIPHON study
2020, Journal of Heart and Lung TransplantationCitation Excerpt :The prognostic value of the noninvasive French approach for transplant-free survival has been demonstrated in registry analyses of newly diagnosed patients,5,8 demonstrating the ability of this approach to identify patients at very low risk. The prognostic value of REVEAL 2.0 has been validated in a registry of predominantly prevalent patients.14 The association between risk and outcome has also been investigated using clinical trial data.
FUNDING/SUPPORT: Funding support for the Pulmonary Hypertension Society of Australia and New Zealand Registry was provided by Actelion Pharmaceuticals, Allied Healthcare, Bayer, GlaxoSmithKline, Novartis, and Pfizer. GlaxoSmithKline provided research funding support for the primary author.