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 multi-centre study

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 or connective tissue disease-associated PAH who had completed emPHasis-10 questionnaires between 2014-17 at 6 UK referral centres were identified. Correlations with exercise capacity and WHO functional class (FC) were assessed, and exploratory risk stratification thresholds were tested. Moderate correlations were seen between emPHasis-10 scores and 6-minute walk distance (r=0.546), incremental shuttle walking distance (r=-0.504) and WHO FC (r=0.497; p all <0.0001). Distribution of emPHasis-10 differed significantly between each WHO FC (p all <0.0001). At multivariate analysis, emPHasis-10, but not WHO FC, was an independent predictor of mortality. In a risk stratification approach, scores of 0-16, 17-33 and 34-50 identified incident patients with oneyear mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO FC III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis10 had improved exercise capacity (p<0.0001), and patients who transitioned risk groups demonstrated similar survival to patients originally in those risk groups. The emPHasis-10 score is an independent prognostic marker in patients with idiopathic or connective tissue disease-associated PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity.