Cross-cultural validation of the Chinese version of the EmPHasis-10 questionnaire in connective tissue disease patients with pulmonary arterial hypertension and its relationship with risk stratification

Abstract

Backgrounds The EmPHasis-10 questionnaire is a disease-specific quality of life (QoL) measurement in patients with pulmonary hypertension. We report the results of cross-cultural validation of the Chinese version of the EmPHasis-10 and its relationship with risk stratification in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). Methods The Emphasis-10 was administered to 75 CTD-PAH patients along with the 36-item Medical Outcomes Study Short Form Survey (SF-36) and EuroQol five dimensions questionnaire (EQ-5D). The diagnosis of PAH was confirmed by right heart catheterization. Demographic and clinical data were obtained. Multivariable logistic regression was conducted based on the low risk profile assessed by a 4-strata risk assessment model (COMPERA 2.0) at follow-up. Results Date from 75 patients with CTD-PAH were analysed. The EmPHasis-10 demonstrated satisfactory reliability (Cronbach α = 0.95) and convergent validity showed by the significant relationship with WHO Functional Class (P = 0.003), SF-36 (P < 0.001) and EQ-5D (P = 0.002). EmPHasis-10 was significantly associated with achieving the low risk profile at 12 months of follow-up (Odds ratio: 0.928, P = 0.029) after adjusting for WHO Functional Class. Conclusion EmPHasis-10 has acceptable reliability and validity in CTD-PAH patients and may serve as an additional parameter in risk stratification.

Relationship between quality of life, fear of disease progression, and coping styles in patients with pulmonary hypertension: A network analysis

“…This can worsen the disease and delay treatment, thus impairing the physiological domain of the patient's QoL. Several studies (Shang et al, 2022;Shi et al, 2022) also found a significant positive correlation between the psychological and physical domains of QoL, which is consistent with the results of this study. In subsequent rehabilitation, patients with PH often have to overcome the psychological stress of waiting for organs, complex surgeries, and finances, often with decreased self-esteem, helplessness, and despair (Chiang et al, 2018;Sobczak-Kaleta et al, 2018) found that as low as 23% of patients with PH would seek psychological support (Muntingh et al, 2017).…”