Prevalence of antiphospholipid (aPL) antibodies among patients with chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis

  • Chun-Yan Cheng
  • Yi-Xin Zhang
  • Gentian Denas
  • Yao Du
  • Zhi-Cheng Jing
  • Vittorio PengoEmail author


How thrombophilia may contribute to the development of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. We searched on PubMed and EMBASE (until 15 April 2018), studies on CTEPH reporting data on inherited or acquired thrombophilia. Starting from 367 articles mentioning the search terms, 347 were excluded mainly as duplicate articles or articles not in English. After reading the full text of remaining articles, ten were excluded for being reviews, editorials, letters or case reports, and two were further removed from the analysis because of the potential selection bias. All the eight considered studies provided the proportion of patients positive for antiphospholipid (aPL) antibodies. The crude rate of aPL in CTPEH patients is 11.8% (95% CI 10.09–13.8%). The meta-analysis considering the weighted mean proportion and 95% confidence intervals (CIs) yields a rate of aPL antibody-positive profile of 12.06% (95% CI 8.12–16.65%) among the patients with CTEPH in the random effects model (I2 76.33%; 95% CI 52.75–88.14%, p = 0.0001). The sensibility analysis confirms the result. No predictors of heterogeneity are found in a meta-regression analysis. Our results suggest that aPL antibodies are frequently associated with CTEPH underlining the need to test for aPL antibodies in young patients with “idiopathic” and “provoked” PE caused by mild provoking risk factors.


Inherited thrombophilia Antiphospholipid antibodies Chronic thromboembolic pulmonary hypertension 



Supported by CAMS Innovation Fund for Medical Sciences (2017-I2M-B&R-02). Capital Characteristic Clinic Project of Beijing Municipal (Z181100001718203). National Science Fund for Distinguished Young Scholars (81425002). The funding sources had no role in the study design, data selection and analysis, quality assessment, data synthesis and interpretation, or the decision to prepare and submit the manuscript for publication.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Statements on human and animal rights

The procedures followed in this study are in accordance with the ethical standards and to our knowledge, no unethical study was included in the meta-analysis. Aggregate data (meta-analyses) are likely to have the same objectives as the original studies.

Informed consent

There is no particular ethical problem regarding the original informed consent of patients.


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Copyright information

© Società Italiana di Medicina Interna (SIMI) 2019

Authors and Affiliations

  1. 1.Cardiology Clinic, Thrombosis Centre, Department of Cardiac Thoracic and Vascular SciencesUniversity of PaduaPaduaItaly
  2. 2.Key Laboratory of Pulmonary Vascular Medicine & FuWai Hospital, State Key Laboratory of Cardiovascular DiseaseChinese Academy Medical Sciences & Peking Union Medical CollegeBeijingPeople’s Republic of China

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