James J Anderson1, Edmund M Lau2, Melanie Lavender3, Raymond Benza4, David S Celermajer5, Nicholas Collins6, Carolyn Corrigan7, Nathan Dwyer8, John Feenstra9, Mark Horrigan10, Dominic Keating11, Fiona Kermeen9, Eugene Kotlyar12, Tanya McWilliams13, Bronwen Rhodes14, Peter Steele15, Vivek Thakkar16, Trevor Williams11, Helen Whitford11, Kenneth Whyte17, Robert Weintraub18, Jeremy P Wrobel19, Anne Keogh12, Geoff Strange20. 1. Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia; Respiratory Department, Sunshine Coast University Hospital, Birtinya, QLD, Australia. Electronic address: james.anderson2@health.qld.gov.au. 2. Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Camperdown, NSW, Australia. 3. Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia. 4. Allegheny General Hospital, Pittsburgh, PA. 5. Sydney Medical School, University of Sydney, Camperdown, NSW, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia. 6. John Hunter Hospital, Newcastle, NSW, Australia. 7. Heart and Lung Transplant Unit, St Vincent's Hospital, Sydney, NSW, Australia. 8. Cardiology Department, Royal Hobart Hospital, Hobart, TAS, Australia. 9. Thoracic Medicine, The Prince Charles Hospital, Brisbane, QLD, Australia. 10. The Austin Hospital, Melbourne, VIC, Australia. 11. Respiratory Medicine, Alfred Hospital, Melbourne, VIC, Australia; Monash University, Melbourne, VIC, Australia. 12. Heart and Lung Transplant Unit, St Vincent's Hospital, Sydney, NSW, Australia; University of New South Wales, Sydney, NSW, Australia. 13. Greenlane Respiratory Services, Auckland City Hospital, Auckland, New Zealand. 14. Department of Respiratory Medicine, Christchurch Hospital, Christchurch, New Zealand. 15. Department of Cardiovascular Services, Royal Adelaide Hospital, Adelaide, SA, Australia. 16. Department of Clinical Medicine, Macquarie University, Macquarie Park, NSW, Australia; Department of Rheumatology, Liverpool Hospital, Liverpool, NSW, Australia. 17. Greenlane Respiratory Services, Auckland City Hospital, Auckland, New Zealand; NZ Respiratory and Sleep Institute, Auckland, New Zealand. 18. Royal Children's Hospital, Melbourne, VIC, Australia; Murdoch Children's Research Institute, Melbourne, VIC, Australia; University of Melbourne, Melbourne, VIC, Australia. 19. Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia; School of Medicine, University of Notre Dame, Fremantle, WA, Australia. 20. School of Medicine, University of Notre Dame, Fremantle, WA, Australia.
Abstract
BACKGROUND: Pulmonary arterial hypertension (PAH) prognosis has improved with targeted therapies; however, the long-term outlook remains poor. Objective multiparametric risk assessment is recommended to identify patients at risk of early morbidity and mortality, and for optimization of treatment. The US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk score is a new model proposed for the follow-up of patients with PAH but has not been externally validated. METHODS: The REVEAL 2.0 risk score was applied to a mixed prevalent and incident cohort of patients with PAH (n = 1,011) from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) Registry. Kaplan-Meier survival was estimated for each REVEAL 2.0 risk score strata and for a simplified three-category (low, intermediate, and high risk) model. Sensitivity analysis was performed on an incident-only cohort. RESULTS: The REVEAL 2.0 model effectively discriminated risk in the large external PHSANZ Registry cohort, with a C statistic of 0.74 (both for full eight-tier and three-category models). When applied to incident cases only, the C statistic was 0.73. The three-category REVEAL 2.0 model demonstrated robust separation of 12- and 60-month survival estimates (all risk category comparisons P < .001). Although the full eight-tier REVEAL 2.0 model separated patients at low, intermediate, and high risk, survival estimates overlapped within some of the intermediate- and high-risk strata. CONCLUSIONS: 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 patients with PAH at follow-up. The simplified three-category model may be preferred for clinical use and for future comparison with other prognostic models. Crown
BACKGROUND:Pulmonary arterial hypertension (PAH) prognosis has improved with targeted therapies; however, the long-term outlook remains poor. Objective multiparametric risk assessment is recommended to identify patients at risk of early morbidity and mortality, and for optimization of treatment. The US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk score is a new model proposed for the follow-up of patients with PAH but has not been externally validated. METHODS: The REVEAL 2.0 risk score was applied to a mixed prevalent and incident cohort of patients with PAH (n = 1,011) from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) Registry. Kaplan-Meier survival was estimated for each REVEAL 2.0 risk score strata and for a simplified three-category (low, intermediate, and high risk) model. Sensitivity analysis was performed on an incident-only cohort. RESULTS: The REVEAL 2.0 model effectively discriminated risk in the large external PHSANZ Registry cohort, with a C statistic of 0.74 (both for full eight-tier and three-category models). When applied to incident cases only, the C statistic was 0.73. The three-category REVEAL 2.0 model demonstrated robust separation of 12- and 60-month survival estimates (all risk category comparisons P < .001). Although the full eight-tier REVEAL 2.0 model separated patients at low, intermediate, and high risk, survival estimates overlapped within some of the intermediate- and high-risk strata. CONCLUSIONS: 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 patients with PAH at follow-up. The simplified three-category model may be preferred for clinical use and for future comparison with other prognostic models. Crown
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