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

Xin Jiang; Yao Du; Chun-Yan Cheng; Gentian Denas; Yu-Ping Zhou; Tao Wu; Yi-Xin Zhang; Zhi-Yan Han; Vittorio Pengo; Zhi-Cheng Jing

doi:10.1055/s-0039-1692428

Thrombosis and Haemostasis, Table of Contents

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^*

¹FuWai 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^*

¹FuWai 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

²Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy

,

Gentian Denas

²Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy

,

Yu-Ping Zhou

¹FuWai 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

¹FuWai 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

¹FuWai 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

³Department of Anaesthesiology, FuWai Hospital, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China

,

Vittorio Pengo

²Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy

,

Zhi-Cheng Jing

¹FuWai 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

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

Full Text

References

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