@article{a9df3cfa27b742008c19d57419989597,
title = "EmPHasis-10 as a measure of health-related quality of life in pulmonary arterial hypertension: Data from PHAR",
abstract = "Introduction: While the performance of the emPHasis-10 (e10) score has been evaluated against limited patient characteristics within the United Kingdom, there is an unmet need for exploring the performance of the e10 score among pulmonary arterial hypertension (PAH) patients in the United States. Methods: Using the Pulmonary Hypertension Association Registry, we evaluated relationships between the e10 score and demographic, functional, haemodynamic and additional clinical characteristics at baseline and over time. Furthermore, we derived a minimally important difference (MID) estimate for the e10 score. Results: We analysed data from 565 PAH (75% female) adults aged mean±SD 55.6±16.0 years. At baseline, the e10 score had notable correlation with factors expected to impact quality of life in the general population, including age, education level, income, smoking status and body mass index. Clinically important parameters including 6-min walk distance and B-type natriuretic peptide (BNP)/N-terminal proBNP were also significantly associated with e10 score at baseline and over time. We generated a MID estimate for the e10 score of −6.0 points (range −5.0-−7.6 points). Conclusions: The e10 score was associated with demographic and clinical patient characteristics, suggesting that health-related quality of life in PAH is influenced by both social factors and indicators of disease severity. Future studies are needed to demonstrate the impact of the e10 score on clinical decision-making and its potential utility for assessing clinically important interventions.",
author = "{PHAR Study Group} and Marissa Borgese and David Badesch and Todd Bull and Murali Chakinala and Teresa DeMarco and Jeremy Feldman and Ford, {H. James} and Dan Grinnan and Klinger, {James R.} and Lena Bolivar and Shlobin, {Oksana A.} and Frantz, {Robert P.} and Sager, {Jeffery S.} and Mathai, {Stephen C.} and Steven Kawut and Leary, {Peter J.} and Gray, {Michael P.} and Popat, {Rita A.} and Zamanian, {Roham T.}",
note = "Funding Information: Acknowledgements: The Pulmonary Hypertension Association Registry (PHAR) is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organisation of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR Conflict of interest: M. Borgese has nothing to disclose. D. Badesch reports grants, personal fees and other (long-term stockholder in Johnson & Johnson) from Actelion/Janssen/Johnson & Johnson, grants and personal fees from Arena/ United Therapeutics/Lung LLC, Acceleron, Complexa, Liquidia and Belleraphon, personal fees from Pfizer, grants from Altavant, outside the submitted work. T. Bull reports grants from Bayer, personal fees from Liquidia, outside the submitted work; and is a member of a clinical trial steering committee for Liquidia. M. Chakinala reports grants and consultancy fees from Actelion, Reata Pharmaceuticals, United Therapeutics, Phase Bio and Acceleron, grants from Medtronic, Eiger Bio Pharmaceuticals, GeNO LLC, GSK, Liquidia, Complexa, Altavant and Trio Health Analytics, consultancy and lecture fees from WedMD LLC (Medscape), consultancy fees from SteadyMed Therapeutics Inc. and Express Scripts Holding Company, grants, personal fees for lectures and consultancy fees from Bayer and Gilead Sciences, outside the submitted work. T. DeMarco serves as a consultant to Actelion, United Therapeutics, Arena and SCOPE/BIAL, and as PI on a multicenter trial sponsored by Acceleron. J. Feldman reports personal fees for consultancy and lectures from United Therapeutics and Actelion, personal fees for consultancy from Acceleron, Bayer and Altavant, outside the submitted work. H.J. Ford reports grants and personal for consultancy fees from United Therapeutics and Actelion/Jannsen, personal fees for consultancy from Bayer, grants from Liquidia, outside the submitted work. D. Grinnan reports grants from Johnson & Johnson, Bayer and United Therapeutics, outside the submitted work. J.R. Klinger has nothing to disclose. L. Bolivar has nothing to disclose. O.A. Shlobin has nothing to disclose. R.P. Frantz has nothing to disclose. J.S. Sager reports personal fees for consultancy, lectures and research from Actelion and United Therapeutics, personal fees for consultancy from Bayer, personal fees for research from Reata and PhaseBio, outside the submitted work. S.C. Mathai reports personal fees for consultancy from Actelion, Arena, Liquidia and United Therapeutics, outside the submitted work; and is a member of the Rare Disease Advisory Panel for the Patient Centered Outcomes Research Institute. S. Kawut reports grants from NIH, non-financial support from ATS, and grants from Actelion, United Therapeutics, Gilead, Lung Biotech, Bayer and Mallinkrodt to the Perelman School of Medicine for CME courses; reports grants and non-financial support from Cardiovascular Medical Research and Education Fund and non-financial support from Pulmonary Hypertension Association; and has served in an advisory capacity (for grant review and other purposes) for United Therapeutics, Akros Pharmaceuticals, GlaxoSmithKline and Complexa, Inc. without financial support or in-kind benefits. P.J. Leary has nothing to disclose. M.P. Gray has nothing to disclose. R.A. Popat has nothing to disclose. R.T. Zamanian reports grants from Actelion, Tenax, PHaware and United Therapeutics, personal fees for consultancy from Morphogen-IX and Vivus, outside the submitted work; and has a patent FK506 for PH issued. Publisher Copyright: Copyright {\textcopyright} ERS 2021",
year = "2021",
doi = "10.1183/13993003.00414-2020",
language = "English",
volume = "57",
journal = "European Respiratory Journal",
issn = "0903-1936",
number = "2",
}