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Title: Retrospective validation of the REVEAL 2.0 Risk Score with the Australian and New Zealand Pulmonary Hypertension Registry Cohort.
Austin Authors: Anderson, James J;Lau, Edmund M;Lavender, Melanie;Benza, Raymond;Celermajer, David S;Collins, Nicholas;Corrigan, Carolyn;Dwyer, Nathan;Feenstra, John;Horrigan, Mark ;Keating, Dominic;Kermeen, Fiona;Kotlyar, Eugene;McWilliams, Tanya;Rhodes, Bronwen;Steele, Peter;Thakkar, Vivek;Williams, Trevor;Whitford, Helen;Whyte, Kenneth;Weintraub, Robert;Wrobel, Jeremy P;Keogh, Anne;Strange, Geoff
Affiliation: Respiratory Department, Sunshine Coast University Hospital, Birtinya, Australia
Sydney Medical School, University of Sydney, Camperdown, Australia
Allegheny General Hospital, Pittsburgh, USA
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia
Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, Australia
School of Medicine, University of Notre Dame, Fremantle, Australia
Department of Cardiovascular Services, Royal Adelaide Hospital, Adelaide, Australia
Thoracic Medicine, The Prince Charles Hospital, Brisbane, Australia
Austin Health
Cardiology Department, Royal Hobart Hospital, Hobart, Australia
Heart and Lung Transplant Unit, St Vincent's Hospital, Sydney, Australia
John Hunter Hospital, Newcastle, Australia
Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, Australia
Greenlane Respiratory Services, Auckland City Hospital, Auckland, New Zealand; NZ Respiratory and Sleep Institute, Auckland, New Zealand
Department of Respiratory Medicine, Christchurch Hospital, Christchurch, New Zealand
Greenlane Respiratory Services, Auckland City Hospital, Auckland, New Zealand
University of New South Wales, Sydney, Australia
Royal Children's Hospital, Melbourne, Australia
Murdoch Children's Research Institute, Melbourne, Australia
University of Melbourne, Melbourne, Australia
Respiratory Medicine, Alfred Hospital, Melbourne, Australia
Monash University, Melbourne, Australia
Macquarie University, Department of Clinical Medicine, Macquarie Park, Australia
Liverpool Hospital, Department of Rheumatology, Liverpool, Australia
Issue Date: 26-Sep-2019
Date: 2020-01
Publication information: Chest 2020; 157(1): 162-172
Abstract: Pulmonary arterial hypertension (PAH) prognosis has improved with targeted therapies although the long-term outlook remains poor. Objective multi-parametric risk assessment is recommended to identify patients at risk of early morbidity and mortality, and for optimization of treatment. The REVEAL 2.0 Risk Score is a new model proposed for the follow-up of PAH patients but has not been externally validated. The REVEAL 2.0 Risk Score was applied to a mixed prevalent and incident cohort of PAH patients (n=1,011) from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) Registry. Kaplan-Meier (KM) survival was estimated for each REVEAL 2.0 Risk Score strata and for a broader three-category (low, intermediate, high-risk) model. Sensitivity analysis was performed on an incident-only cohort. The REVEAL 2.0 model effectively discriminated risk in the large external PHSANZ registry cohort, with c-statistic 0.74 (both for full 8-tier and three-category models). When applied to incident cases only, c-statistic was 0.73. The three-category REVEAL 2.0 model demonstrated robust separation of 12 and 60-months survival estimates (all risk categories comparisons p<0.001). Although the full 8-tier REVEAL 2.0 model separated low, intermediate and high-risk patients, survival estimates overlapped within some of the intermediate and high-risk strata. The REVEAL 2.0 risk score was validated in a large external cohort from the PHSANZ Registry. The REVEAL 2.0 model can be applied for risk assessment of PAH patients at follow-up. The simplified 3-category model may be preferred for clinical use and for future comparison with other prognostic models.
DOI: 10.1016/j.chest.2019.08.2203
Journal: Chest
PubMed URL: 31563497
Type: Journal Article
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