ArticleThe European respiratory journal2021
EmPHasis-10 health-related quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multicentre study.
Article in The European respiratory journal, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 4 of them syntheses that pooled it.
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Who cites it
34 citing papers in PubMed, 4 syntheses or guidelines pooled it.
- Quality of life in patients with pulmonary arterial hypertension: A systematic review and meta-analysis.Health and quality of life outcomes · 2026Pooled it
- Selection of patient-reported outcome measures in pulmonary arterial hypertension clinical trials: a systematic review, meta-analysis and health-related quality of life framework.European respiratory review : an official journal of the European Respiratory Society · 2025Pooled it
- Prognostic Value of Serial Risk Stratification in Adult and Pediatric Pulmonary Arterial Hypertension: A Systematic Review.Journal of the American Heart Association · 2024Pooled it
- Palliative care in pulmonary hypertension associated with congenital heart disease: systematic review and expert opinion.ESC heart failure · 2021Pooled it
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- Patient Perspectives on Palliative Care in Pulmonary Arterial Hypertension in the United States.Pulmonary circulation · 2026Article
- Validation Analysis of the Polish-Translated Version of EmPHasis-10 Health-Related Quality of Life Questionnaire in Patients with Pulmonary Arterial Hypertension.Journal of clinical medicine · 2026Article
- Validity and reliability testing of the Thai version of the emPHasis-10 questionnaire for patients with pulmonary arterial hypertension.Journal of patient-reported outcomes · 2026Article
- The PAH patient's perspective.International journal of cardiology. Congenital heart disease · 2025Review
- Validation of the Pulmonary Arterial Hypertension-Symptoms and Impact for Clinical Use (SYMPACT-CP): a qualitative interview study.BMC pulmonary medicine · 2025Article
- Relationships Among the EmPHasis-10 Questionnaire, the Simplified Four-Strata Risk Assessment Tool, and Echocardiographic Parameters in Patients with Precapillary Pulmonary Hypertension.Journal of clinical medicine · 2024Article
- Risk stratification and treatment goals in pulmonary arterial hypertension.The European respiratory journal · 2024Review
- Management of pulmonary hypertension in special conditions.The European respiratory journal · 2024Review
- Association of risk assessment at diagnosis with healthcare resource utilization and health-related quality of life outcomes in pulmonary arterial hypertension.Pulmonary circulation · 2024Article
- Article
- Article
- Exploratory and confirmatory factor analysis of emPHasis-10: The health-related quality-of-life measure in pulmonary hypertension.Pulmonary circulation · 2024Article
- Assessing quality of life in pulmonary arterial hypertension: An independent prognostic marker.Pulmonary circulation · 2024Article
- Assessing the clinical benefit, safety, and patient-reported outcomes with the use of the PAHcare™ digital platform in pulmonary arterial hypertension: a pilot study.Frontiers in public health · 2024Article
- Pulmonary Hypertension: Intensification and Personalization of Combination Rx (PHoenix): A phase IV randomized trial for the evaluation of dose-response and clinical efficacy of riociguat and selexipag using implanted technologies.Pulmonary circulation · 2024Article
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Abstract
Health-related quality of life (HRQoL) scores assess symptom burden in pulmonary arterial hypertension (PAH) but data regarding their role in prognostication and risk stratification are limited. We assessed these relationships using the emPHasis-10 HRQoL measure.1745 patients with idiopathic PAH (IPAH), drug-induced PAH (DPAH), heritable PAH (HPAH) (collectively "(I/D/H)PAH"), or connective tissue disease-associated PAH (CTD-PAH), who had completed emPHasis-10 questionnaires at one of six UK referral centres between 2014 and 2017, were identified. Correlations with exercise capacity and World Health Organization (WHO) functional class were assessed, and exploratory risk stratification thresholds were tested.Moderate correlations were seen between emPHasis-10 scores and 6-min walk distance (r=-0.546), incremental shuttle walk distance (r=-0.504) and WHO functional class (r=0.497) (all p<0.0001). Distribution of emPHasis-10 score differed significantly between each WHO functional class (all p<0.0001). On multivariate analysis, emPHasis-10 score, but not WHO functional class, was an independent predictor of mortality. In a risk stratification approach, scores of 0-16, 17-33 and 34-50 identified incident patients with 1-year mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO functional class III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis-10 scores had improved exercise capacity (p<0.0001) and patients who transitioned between risk groups demonstrated similar survival to patients originally in those risk groups.The emPHasis-10 score is an independent prognostic marker in patients with (I/D/H)PAH or CTD-PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity.
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