Quality of life in ambulatory pulmonary arterial hypertension in connective tissue diseases and its relationship with risk stratification

Yue Shi; Yanling Meng; Xingbei Dong; Yang Liu; Yongtai Liu; Jinzhi Lai; Zhuang Tian; Jiuliang Zhao; Jinmin Peng; Qian Wang; Mengtao Li; Xiaofeng Zeng

doi:10.1177/20458940211029899

Quality of life in ambulatory pulmonary arterial hypertension in connective tissue diseases and its relationship with risk stratification

Pulm Circ. 2021 Jul 11;11(3):20458940211029899. doi: 10.1177/20458940211029899. eCollection 2021 Jul-Sep.

Authors

Affiliations

¹ Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.
² Medical Intensive Care Unit, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
³ Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.

Abstract

The Pulmonary Arterial Hypertension Symptoms and Impact Questionnaire (PAH-SYMPACT) is a PAH-specific patient-reported outcome scale assessing patients' quality of life 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). In addition, 75 patients with CTD-PAH confirmed by right heart catheterization 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 status in 6-12 months after completing the questionnaires, defined as an integrated outcome. Participants' mean age was 36.4 ± 11.9 years and the mean pulmonary arterial pressure was 38.9 ± 13.67 mmHg. The reliability of the PAH-SYMPACT domains ranged from 0.83 to 0.88. Results of factor analysis basically conformed the original PAH-SYMPACT. The treatment goal achievement (TGA) status in 6-12 months was significantly associated with physical impacts scores (odds ratio: 0.180, 95% confidence interval: 0.036-0.908, P=0.038). The Chinese version of PAH-SYMPACT is a reliable measurement to evaluate quality of life in CTD-PAH patients and is also a potential predictor of patient's condition change in routine clinical practice.

Keywords: CTD-PAH; PAH-SYMPACT; connective tissue disease; pulmonary arterial hypertension; quality of life.