Clinical features and survival in Takayasu’s arteritis-associated pulmonary hypertension: a nationwide study

Author:

Jiang Xin1,Zhu Yong-Jian1,Zhou Yu-Ping1,Peng Fu-Hua2ORCID,Wang Lan3,Ma Wei4,Cao Yun-Shan5,Pan Xin6,Zhang Gang-Cheng7,Zhang Feng8,Fan Fen-Ling9,Wu Bing-Xiang10,Huang Wei11ORCID,Yang Zhen-Wen12,Hong Cheng13,Li Meng-Tao14,Wang Yi-Ning15,Xu Xi-Qi1,Wang Duo-Lao16ORCID,Zhang Shu-Yang1,Jing Zhi-Cheng1ORCID

Affiliation:

1. Department of Cardiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1, Shuaifuyuan, Dongcheng District, Beijing 100730, China

2. Department of Pulmonary Vascular Disease and Thrombosis Medicine, FuWai Hospital, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167, Beilishi Road, Xicheng Distirct, Beijing 100037, China

3. Department of Cardio-Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, No. 507, Zheng Min Road, Yangpu District, Shanghai 200433, China

4. Department of Cardiology, Peking University First Hospital, Peking University, No. 8, Xishiku Street, Xicheng District, Beijing 100034, China

5. Department of Cardiology, Gansu Provincial Hospital, No. 204, Donggang West Road, Chengguan District, Lanzhou 730000, China

6. Department of Cardiology, Shanghai Chest Hospital, Shanghai Jiao Tong University, No. 241, West Huaihai Road, Xuhui District, Shanghai 200030, China

7. Congenital Heart Disease Center, Wuhan Asia Heart Hospital, No. 753, Jinghan Ave, Jianghan District, Wuhan 430022, China

8. Department of Respiratory, General Hospital of Xinjiang Military Region, No. 359, Youhao North Road, Saybak District, Urumqi 830000, China

9. Department of Cardiology, The First Affiliated Hospital of Xi’an Jiaotong University, Xi’an Jiaotong University, No. 277, Yanta West Road, Yanta District, Xi’an 710061, China

10. Department of Cardiology, The Second Affiliated Hospital of Harbin Medical University, Harbin Medical University, No. 246, Xuefu Road, Nangang District, Harbin 150001, China

11. Department of Cardiology, The First Affiliated Hospital of Chongqing Medical University, Chongqing Medical University, No. 1, Youyi Road, Yuzhong District, Chongqing 400016, China

12. Department of Cardiology, Tianjin Medical University General Hospital, Tianjin Medical University, No. 154, Anshan Road, Heping District, Tianjin 300052, China

13. State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University, No. 151, Yanjiang West Road, Yuexiu District, Guangzhou 510120, China

14. Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1, Shuaifuyuan, Dongcheng District, Beijing 100730, China

15. Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 1, Shuaifuyuan, Dongcheng District, Beijing 100730, China

16. Department of Clinical Sciences, Liverpool School of Tropical Medicine, Pembroke Place, Liverpool L3 5QA, UK

Abstract

Abstract Aims This study aimed to assess the clinical characteristics and long-term survival outcome in patients with Takayasu’s arteritis-associated pulmonary hypertension (TA-PH). Methods and results We conducted a nationally representative cohort study of TA-PH using data from the National Rare Diseases Registry System of China. Patients with pulmonary artery involvement who fulfilled the diagnostic criteria of Takayasu’s arteritis and pulmonary hypertension were included. The primary outcome was the time from diagnosis of TA-PH to the occurrence of all-cause death. Between January 2007 and January 2019, a total of 140 patients were included, with a mean age of 41.4 years at diagnosis, and a female predominance (81%). Patients with TA-PH had severely haemodynamic and functional impairments at diagnosis. Significant improvements have been found in N-terminal pro-B-type natriuretic peptide (NT-proBNP) and haemodynamic profiles in patients with TA-PH receiving drugs approved for pulmonary arterial hypertension. The overall 1-, 3-, and 5-year survival rates in TA-PH were 94.0%, 83.2%, and 77.2%, respectively. Predictors associated with an increased risk of all-cause death were syncope [adjusted hazard ratio (HR) 5.38 (95% confidence interval 1.77–16.34), P = 0.003], NT-proBNP level [adjusted HR 1.04 (1.03–1.06), P < 0.001], and mean right atrial pressure [adjusted HR 1.07 (1.01–1.13), P = 0.015]. Conclusion Patients with TA-PH were predominantly female and had severely compromised haemodynamics. More than 80% of patients in our cohort survived for at least 3 years. Medical treatment was based on investigators’ personal opinions, and no clear risk-to-benefit ratio can be derived from the presented data.

Funder

National Key Research and Development Program of China

Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences

Publisher

Oxford University Press (OUP)

Subject

Cardiology and Cardiovascular Medicine

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