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Antiphospholipid antibodies in chronic thromboembolic pulmonary hypertension

  • Author Footnotes
    1 These two authors share the first authorship.
    Rui Zhu
    Footnotes
    1 These two authors share the first authorship.
    Affiliations
    Department of Endocrinology, Sichuan Academy of Medical Sciences & Sichuan Provincial People's Hospital, Chengdu, China
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  • Author Footnotes
    1 These two authors share the first authorship.
    Gang-Yi Cheng
    Footnotes
    1 These two authors share the first authorship.
    Affiliations
    Department of Cardiac Surgery, The First Affiliated Hospital of XiaMen University, XiaMen, China
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  • Gentian Denas
    Affiliations
    Department of Cardio-Thoracic-Vascular Sciences and Public Health, Thrombosis Research Laboratory, University of Padua, Padua, Italy
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  • Vittorio Pengo
    Correspondence
    Corresponding author at: Thrombosis Research Laboratory, University of Padova, Campus Biomedico, ‘Pietro d'Abano,’ Via Orus 2/B, Padova 35129, Italy.
    Affiliations
    Department of Cardio-Thoracic-Vascular Sciences and Public Health, Thrombosis Research Laboratory, University of Padua, Padua, Italy

    Arianna Foundation on Anticoagulation, Bologna, Italy
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  • Author Footnotes
    1 These two authors share the first authorship.
Published:January 13, 2023DOI:https://doi.org/10.1016/j.ejim.2023.01.012

      Highlights

      • CTEPH is a severe condition often following an episode of PE.
      • APL are present in around 12% of patients with CTEPH.
      • APL are a risk factor for recurrent PE that in turn is a risk factor for CTEPH.
      • APL should be tested in all the young patients (<50yrs) with unprovoked PE.
      • Pulmonary endarterectomy and warfarin are the mainstays of treatment.

      Abstract

      Acquired thrombophilia and in particular the presence of antiphospholipid antibodies (aPL) may play an important role in the development of chronic thromboembolic pulmonary hypertension (CTEPH). Young patients suffering from an episode of unprovoked pulmonary embolism (PE), or PE provoked by mild risk factors, should be tested for aPL. In case of a positive result, they should be closely followed up and lifelong anticoagulant treatment should be considered. Indeed, aPL-induced thrombophilia may favor PE recurrence with the consequence of possible CTEPH development. The aPL profiles play an important role in this pathway. Patients with PE and triple positivity (lupus anticoagulant, LAC, anti-cardiolipin, aCL, and anti-β2-glycoprotein I, aβ2GPI) are at the highest risk of recurrence and deserve maximum protection by anticoagulant treatment with warfarin.

      Keywords

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