EXPRESS: Quality of life in ambulatory pulmonary arterial hypertension in connective tissue diseases and its relationship with risk stratification
The Pulmonary Arterial Hypertension Symptoms and Impact Questionnaire (PAH-SYMPACT) is a PAH-specific patient-reported outcome scale assessing patientsâ quality of life (QoL) from four aspects: cardiopulmonary symptoms, cardiovascular symptoms, physical impacts and cognitive/emotional impacts. This study aimed to validate the Chinese version of PAH-SYMPACT and explore its relationship with risk stratification in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).75 patients with CTD-PAH confirmed by right heart catheterization (RHC) were invited to complete questionnaires including PAH-SYMPACT, the 36-item Medical Outcomes Study Short Form Survey (SF-36) and EuroQol five dimensions questionnaire (EQ-5D). The demographic, clinical, laboratory and treatment data were collected. The endpoint was treatment goal achievement (TGA) status in 6-12 months after completing the questionnaires, defined as an integrated outcome. Participantsâ mean age was 36.4±11.9 years and the mean pulmonary arterial pressure (mPAP) was 38.9±13.67 mmHg. The reliability of the PAH-SYMPACT domains ranged from 0.83-0.88. Results of factor analysis basically conformed the original PAH-SYMPACT. The PGA status in 6-12 months was significantly associated with physical impacts scores (OR:0.180, 95% CI: 0.036-0.908, P=0.038). The Chinese version of PAH-SYMPACT is a reliable measurement to evaluate QoL in CTD-PAH patients and is also a potential predictors of patientâs condition change in routine clinical practice.