EmPHasis-10 as a measure of health-related quality of life in pulmonary arterial hypertension: data from PHAR

Marissa Borgese; David Badesch; Todd Bull; Murali Chakinala; Teresa DeMarco; Jeremy Feldman; H James Ford; Dan Grinnan; James R Klinger; Lena Bolivar; Oksana A Shlobin; Robert P Frantz; Jeffery S Sager; Stephen C Mathai; Steven Kawut; Peter J Leary; Michael P Gray; Rita A Popat; Roham T Zamanian; PHAR Study Group

doi:10.1183/13993003.00414-2020

EmPHasis-10 as a measure of health-related quality of life in pulmonary arterial hypertension: data from PHAR

Eur Respir J. 2021 Feb 25;57(2):2000414. doi: 10.1183/13993003.00414-2020. Print 2021 Feb.

Authors

Marissa Borgese¹, David Badesch², Todd Bull², Murali Chakinala³, Teresa DeMarco⁴, Jeremy Feldman⁵, H James Ford⁶, Dan Grinnan⁷, James R Klinger⁸, Lena Bolivar⁹, Oksana A Shlobin¹⁰, Robert P Frantz¹¹, Jeffery S Sager¹², Stephen C Mathai¹³, Steven Kawut¹⁴, Peter J Leary¹⁵, Michael P Gray¹⁶, Rita A Popat¹, Roham T Zamanian^{17

18}; PHAR Study Group

Affiliations

¹ Dept of Health Research and Policy, Stanford University School of Medicine, Stanford, CA, USA.
² Division of Pulmonary and Critical Care Medicine, University of Colorado, Denver, CO, USA.
³ Division of Pulmonary and Critical Care Medicine, Washington University at Barnes-Jewish, Saint Louis, MO, USA.
⁴ Division of Cardiology, University of California San Francisco, San Francisco, CA, USA.
⁵ Arizona Pulmonary Specialists, Phoenix, AZ, USA.
⁶ Division of Pulmonary and Critical Care Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
⁷ Division of Pulmonary and Critical Care Medicine, Virginia Commonwealth University Medical Center, Richmond, VA, USA.
⁸ Division of Pulmonary and Critical Care Medicine, Brown University, Providence, RI, USA.
⁹ Patient representative, USA.
¹⁰ Inova Fairfax Hospital, Falls Church, VA, USA.
¹¹ Division of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, MN, USA.
¹² Cottage Pulmonary Hypertension Center, Santa Barbara, CA, USA.
¹³ Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, Baltimore, MD, USA.
¹⁴ Division of Pulmonary and Critical Care Medicine, University of Pennsylvania, Philadelphia, PA, USA.
¹⁵ Division of Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA, USA.
¹⁶ Pulmonary Hypertension Association, Silver Spring, MD, USA.
¹⁷ Division of Pulmonary and Critical Care Medicine, Stanford University School of Medicine, Stanford, CA, USA Zamanian@stanford.edu.
¹⁸ Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine, Stanford, CA, USA.

PMID: 33243844
DOI: 10.1183/13993003.00414-2020

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.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Aged
Familial Primary Pulmonary Hypertension
Female
Humans
Hypertension, Pulmonary*
Male
Middle Aged
Natriuretic Peptide, Brain
Peptide Fragments
Pulmonary Arterial Hypertension*
Quality of Life
United Kingdom

Substances

Peptide Fragments
Natriuretic Peptide, Brain

Grants and funding

K24 HL103844/HL/NHLBI NIH HHS/United States