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Thromb Haemost 2019; 119(09): 1403-1408
DOI: 10.1055/s-0039-1692428
Coagulation and Fibrinolysis
Georg Thieme Verlag KG Stuttgart · New York

Antiphospholipid Syndrome in Chronic Thromboembolic Pulmonary Hypertension: A Well-Defined Subgroup of Patients

Xin Jiang   *
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Yao Du   *
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Chun-Yan Cheng
2   Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
,
Gentian Denas
2   Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
,
Yu-Ping Zhou
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Tao Wu
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Yi-Xin Zhang
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Zhi-Yan Han
3   Department of Anaesthesiology, FuWai Hospital, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Vittorio Pengo
2   Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
,
Zhi-Cheng Jing 
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
› Author Affiliations Funding This study was supported by Capital Clinical Specialty Project of Beijing Municipal (Z181100001718203 and Z171100001017195). CAMS Innovation Fund for Medical Sciences (CIFMS) (2016-I2M-1–002, 2017-I2M-3–003, 2017-I2M-2–001, and 2017-I2M-B&R-02). National Science Fund for Distinguished Young Scholars (81425002). The National Key Research and Development Program of China (2016YFC0901502).
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Publication History

20 February 2019

01 May 2019

Publication Date:
21 June 2019 (online)

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Abstract

Antiphospholipid syndrome (APS) is an acquired thrombophilia with an uncertain role in the development of chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to assess the association of APS with the clinical phenotype of CTEPH. We retrospectively reviewed data of CTEPH patients referred to our center. Clinical, angiographic, and hemodynamic data were available for all patients. APS was diagnosed in the presence of one or more positive antiphospholipid (aPL) tests confirmed more than 12 weeks apart. Data were compared between APS-positive and APS-negative patients. From May 2013 to December 2018, 297 patients with CTEPH were enrolled. Twenty-three (7.7%) were positive for laboratory tests exploring aPL antibodies. Among them, 17 patients (74%) had a triple positive aPL profile. When compared with the APS-negative group, APS patients were significantly younger (30.0 ± 11.1 vs. 55.6 ± 12.9 years, p < 0.0001), had more frequently a history of pulmonary embolism (95.6% vs. 65.7%, p = 0.003), and had more frequently associated autoimmune disease (43.5% vs. 2.9%, p < 0.0001). In APS-positive patients, pulmonary artery lesions were more proximal and hemodynamic profiles were less compromised. Our results show that patients with APS are a unique group of CTEPH patients with well-defined clinic and hemodynamic characteristics.

Keywords

antiphospholipid antibodies - chronic thromboembolic pulmonary hypertension - antiphospholipid syndrome

* Xin Jiang and Yao Du equally contributed to this article.