Predicting Survival in Pulmonary Arterial Hypertension: Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)
Abstract
Background— Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use. Methods and Results— Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance >32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men >60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique. Conclusions— We identified key predictors of survival based on the patient’s most recent evaluation and formulated a contemporary prognostic equation. Use of this tool may allow the individualization and optimization of therapeutic strategies. Serial follow-up and reassessment are warranted. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00370214.
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The REVEAL subsequent score uses three non-modifiable and nine modifiable weighted baseline variables and five strata to predict one-year survival and outcomes up to five years.
“Using a comprehensive battery of three non-modifiable and nine modifiable weighted variables measured at baseline, the RSS incorporates five strata to predict one-year survival, as well as long term outcome data up to five years.”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [283]
The Prognostic Equation section defines five risk strata for predicted one-year survival, supporting that part of the claim. The paper instead describes a weighted model with 19 factors, not a specified set of three non-modifiable and nine modifiable variables. The Survival section reports follow-up of at most 731 days; this paper does not establish outcomes up to five years. (LLM, conf=0.90)
Experimental — accuracy not yet measuredThe REVEAL equation and REVEAL subsequent score were derived from 2,716 patients with incident or prevalent pulmonary arterial hypertension or associated pulmonary arterial hypertension from 54 US centers.
“The REVEAL equation and REVEAL subsequent score (RSS) were derived from a cohort of 2,716 patients with incident and prevalent PAH, or associated PAH, from 54 centers across the US.(283)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [283]
The Discussion reports that a prognostic equation was developed from 2,716 patients, and REVEAL Study Design identifies 54 US facilities. Characteristics at Enrollment includes newly and previously diagnosed patients and associated PAH subgroups. The paper does not report deriving the subsequent REVEAL score (RSS) from this cohort. Page numbers are unavailable in our copy. (LLM, conf=0.88)
Experimental — accuracy not yet measuredThe two most frequently used pulmonary arterial hypertension risk assessment models are REVEAL 2.0 and the 2015 ESC/ERS model.
“The two most frequently used risk assessment models are the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) 2.0 and the 2015 European Society of Cardiology/European Respiratory Society (ESC/ERS) model.(283, 284)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [283]
The Discussion (page not provided) says this paper developed a REVEAL prognostic equation for pulmonary arterial hypertension, supporting the REVEAL connection. It does not identify that equation as REVEAL 2.0 or establish that REVEAL 2.0 and the 2015 ESC/ERS model are the two most frequently used models. (LLM, conf=0.88)
Experimental — accuracy not yet measured
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- Authors
- Benza, R. L., Miller, D. P., Gomberg-Maitland, M., Frantz, R. P., Foreman, A. J., Coffey, C. S., Frost, A., Barst, R. J., Badesch, D. B., Elliott, C. G., Liou, T. G., & McGoon, M. D.
- Affiliations
- Mayo Clinic (US)
- From the Allegheny General Hospital, Pittsburgh, Pa (R.L.B.); ICON Clinical Research, San Francisco, Calif (D.P.M., A.J.F.); University of Chicago Medical Center, Chicago, Ill (M.G.-M.); Mayo Clinic, Rochester, Minn (R.P.F., M.D.M.); University of Alabama at Birmingham (C.S.C.); Baylor College of Medicine, Houston, Tex (A.F.); Columbia University College of Physicians and Surgeons, New York, NY (R.J.B.); University of Colorado, Denver (D.B.B.); Intermountain Medical Center, Salt Lake City, Utah (C.G...
- ORCID
- Venue
- Circulation
- Published
- 2010-07-13
- Volume
- 122
- Issue
- 2
- Pages
- 164-172
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W1984693219
- Semantic Scholar
- f47deffdd734b91fe8e3625af12486c97303cb25
- Cited by
- 1,547
- References
- 25
- Citations by year
- 2026: 33 · 2025: 65 · 2024: 74 · 2023: 93 · 2022: 96 · 2021: 114 · 2020: 93 · 2019: 97 · 2018: 110 · 2017: 123 · 2016: 83 · 2015: 119 · 2014: 109 · 2013: 115 · 2012: 120
- FWCI
- 89.09
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Cystic Fibrosis Foundation
- Intermountain Healthcare
- Pfizer
- Gilead Sciences
- United Therapeutics Corporation
- National Institutes of Health
- Genentech
- National Heart, Lung, and Blood Institute
- Actelion Pharmaceuticals
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Genetics › Vascular Anomalies and Treatments
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Keywords
- pulmonary arterial hypertension, survival prediction, prognostic factors, mortality, pulmonary vascular resistance, portal hypertension, functional class, family history (medicine), connective tissue diseases, brain natriuretic peptide, diffusing capacity for carbon monoxide, pericardial effusion, REVEAL registry
- MeSH terms
- Adult, Aged, Blood Pressure, Carbon Monoxide, Female, Heart Rate, Humans, Hypertension, Pulmonary, Male, Middle Aged, Prospective Studies, Registries, Vascular Resistance, Survival Rate, Multivariate Analysis, Disease-Free Survival, Natriuretic Peptide, Brain, Renal Insufficiency
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