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Acquired von Willebrand syndrome in patients treated with veno-arterial extracorporeal membrane oxygenation

  • Toshihiro TamuraEmail author
  • Hisanori Horiuchi
  • Yuki Obayashi
  • Masayuki Fuki
  • Miyako Imanaka
  • Maiko Kuroda
  • Shunsuke Nishimura
  • Masashi Amano
  • Jiro Sakamoto
  • Yodo Tamaki
  • Soichiro Enomoto
  • Makoto Miyake
  • Hirokazu Kondo
  • Chisato Izumi
  • Yoshihisa Nakagawa
Original Article
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Abstract

Veno-arterial extracorporeal membrane oxygenation (VA ECMO) is a powerful device for treatment of patients with life-threatening heart failure. Although bleeding is often associated with VA ECMO and sometimes results in a fatal outcome, its precise causes remain unknown. On the other hand, excessive high shear stress in the cardiovascular system causes acquired von Willebrand syndrome (aVWS), characterized by loss of von Willebrand factor (vWF) large multimers. vWF large multimers of five consecutive patients treated with VA ECMO were quantitatively evaluated using the vWF large multimer indices, defined as the ratio of the large multimer ratio of a patient to that of a healthy subject analyzed simultaneously. All 5 patients exhibited oozing type of bleeding at the skin insertion sites under treatment with PCPS at flow rates of 2.5–3.0 l/min/m2, including two severe cases of bleeding; one patient had massive gastrointestinal bleeding and another had hemothorax. Their vWF large multimer indices were 20.8, 28.8, 27.6, 51.0, and 31.0% (means 31.8 ± 11.4%). Surprisingly, these values are much lower than those observed in severe aortic stenosis reported previously by us (Tamura et al. in J Atheroscler Thromb 22:1115–1123, 2015), where vWF multimer indices in 31 severe aortic stenosis patients with peak pressure gradient through the aortic valves of 85.1 ± 29.4 mmHg were 75.0 ± 21.7% (p < 0.0001), indicating that much higher grade of aVWS occurred in patients with VA ECMO than severe aortic stenosis patients. All the 5 patients treated with VA ECMO developed aVWS that was much more severe than in patients with severe aortic stenosis.

Keywords

VA ECMO Bleeding Acquired von Willebrand syndrome Aortic stenosis Large VWF multimer index 

Notes

Acknowledgements

This study was partly supported by a Health and Labour Sciences Research Grant for the ‘Research on rare and intractable diseases’ from the Ministry of Health, Labour and Welfare, Japan, to HH and YN, and grants from the SENSHIN Medical Research Foundation and the Suzuken Memorial Foundation to HH.

Funding

None.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Japanese Association of Cardiovascular Intervention and Therapeutics 2019

Authors and Affiliations

  • Toshihiro Tamura
    • 1
    Email author
  • Hisanori Horiuchi
    • 2
  • Yuki Obayashi
    • 1
  • Masayuki Fuki
    • 1
  • Miyako Imanaka
    • 1
  • Maiko Kuroda
    • 1
  • Shunsuke Nishimura
    • 1
  • Masashi Amano
    • 1
  • Jiro Sakamoto
    • 1
  • Yodo Tamaki
    • 1
  • Soichiro Enomoto
    • 1
  • Makoto Miyake
    • 1
  • Hirokazu Kondo
    • 1
  • Chisato Izumi
    • 1
  • Yoshihisa Nakagawa
    • 1
  1. 1.Department of CardiologyTenri HospitalTenriJapan
  2. 2.Department of Molecular and Cellular Biology, Institute of Development, Aging and CancerTohoku UniversitySendaiJapan

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