| Literature DB >> 32631835 |
Robert A Lewis1,2, Iain Armstrong1, Carmel Bergbaum3, Melanie J Brewis4, John Cannon5, Athanasios Charalampopoulos1, A Colin Church4, J Gerry Coghlan6, Rachel J Davies7, Konstantinos Dimopoulos3, Charlie Elliot1, J Simon R Gibbs7, Wendy Gin-Sing7, Gulam Haji7, Abdul G Hameed1,2, Luke S Howard7, Martin K Johnson4, Aleksander Kempny3, David G Kiely1,2, Francesco Lo Giudice7, Colm McCabe3, Andrew J Peacock4, Oyinkansola Peleyeju6, Joanna Pepke-Zaba5, Gary Polwarth5, Laura Price3, Ian Sabroe1,2, Benjamin E Schreiber6, Karen Sheares5, Dolores Taboada5, A A Roger Thompson1,2, Mark R Toshner5, Ivy Wanjiku6, S John Wort3, Janelle Yorke8, Robin Condliffe9.
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.Entities:
Year: 2021 PMID： 32631835 PMCID： PMC7905834 DOI： 10.1183/13993003.00124-2020
Source DB: PubMed Journal: Eur Respir J ISSN： 0903-1936 Impact factor: 16.671