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Superior Vena Cava Thrombosis

  • Etienne Rivière
    Correspondence
    Requests for reprints should be addressed to Etienne Riviere, Department of Internal Medicine and Infectious Diseases, Haut-Leveque Hospital, University Hospital of Bordeaux, 33604 Pessac, France.
    Affiliations
    Department of Internal Medicine and Infectious Diseases, Haut-Leveque Hospital, University Hospital of Bordeaux, Pessac, France

    University of Bordeaux, Inserm U1034, Pessac, France
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  • Claire Bazin
    Affiliations
    Radiology Department, Haut-Leveque Hospital, University Hospital of Bordeaux, Pessac, France
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      To the Editor:
      A 60-year old man presented to our unit for iterative episodes of lipothymia and prominent superficial collateral venous circulation of the trunk (Figure A) after he was lost to follow-up for 4 years. He received 2 successive bone marrow transplantations in 2012 for dendritic cell leukemia. Heterozygous prothrombin G20210A mutation was found after a superior vena cava thrombosis from the implantable port occurred in 2015 with a recurrence in 2016 after anticoagulation was stopped. In 2017, a 12-cm long stent was implanted in the superior vena cava, and tinzaparin was given. The newly performed computed tomography scan evidenced a complete superior vena cava stent thrombosis (Figure B, yellow arrow) with important derivations joining the central venous circulation through femoral veins (Figure C, blue arrows) while he was still receiving tinzaparin. No other underlying cause was found. Rivaroxaban was started and the patient was discharged.
      Figure
      Figure(A) Superior vena cava syndrome with a wide superficial collateral venous circulation drawing blood from the upper body; (B) three-dimensional computed tomography reconstruction in coronal view evidencing the stent thrombosis in the superior vena cava (yellow arrow); (C) coronal view turned three-quarters to the right showing the collateral venous circulation of the upper body joining the central venous return through femoral veins (blue arrows).
      Superior vena cava syndrome is now rare and due to compression or thrombosis in 70% of cases linked to mediastinal malignancies (small cell carcinoma of the lung, lymphoma or metastasis). However, the remaining benign causes should be kept in mind (pacemaker wires, stents or catheters).
      • Rice TW
      • Rodriguez RM
      • Light RW.
      The superior vena cava syndrome: clinical characteristics and evolving etiology.
      Clinical manifestations are mostly face, neck or upper extremity swelling, dyspnea/orthopnea, cough, headache or conjunctival suffusion.
      Prolonged anticoagulation is still debated in patients harboring heterozygous prothrombin G20210A mutation, as is the use of direct oral anticoagulants (DOAC).
      • Elsebaie MAT
      • van Es N
      • Langston A
      • Büller HR
      • Gaddh M.
      Direct oral anticoagulants in patients with venous thromboembolism and thrombophilia: a systematic review and meta-analysis.
      Indeed, these patients have a low risk of thrombophilia, and data are scarce to formally prescribe DOAC as no randomized control trial has been specifically conducted in patients with low-risk thrombophilia, even though a recent work found that, compared with vitamin K antagonists, DAOCs had equivalent efficacy and safety.
      • Valanejad SM
      • Davis KA.
      Direct oral anticoagulants in select patients with hypercoagulable disorders.
      DOACs can be proposed after a recurrent thrombotic episode despite adequate anticoagulation with vitamin K antagonist or heparin, like our patient. Additional acquired thrombotic risk factors should trigger the discussion of a prolonged anticoagulation in patients with congenital thrombotic risk factors, such as any implantable material.

      References

        • Rice TW
        • Rodriguez RM
        • Light RW.
        The superior vena cava syndrome: clinical characteristics and evolving etiology.
        Medicine (Baltimore). 2006; 85: 37-42
        • Elsebaie MAT
        • van Es N
        • Langston A
        • Büller HR
        • Gaddh M.
        Direct oral anticoagulants in patients with venous thromboembolism and thrombophilia: a systematic review and meta-analysis.
        J Thromb Haemost. 2019; 17: 645-656
        • Valanejad SM
        • Davis KA.
        Direct oral anticoagulants in select patients with hypercoagulable disorders.
        Ann Pharmacother. 2021; 55: 891-901