Acquired von Willebrand syndrome in patients treated with veno-arterial extracorporeal membrane oxygenation
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ORIGINAL ARTICLE
Acquired von Willebrand syndrome in patients treated with veno‑arterial extracorporeal membrane oxygenation Toshihiro Tamura1 · Hisanori Horiuchi2 · Yuki Obayashi1 · Masayuki Fuki1 · Miyako Imanaka1 · Maiko Kuroda1 · Shunsuke Nishimura1 · Masashi Amano1 · Jiro Sakamoto1 · Yodo Tamaki1 · Soichiro Enomoto1 · Makoto Miyake1 · Hirokazu Kondo1 · Chisato Izumi1 · Yoshihisa Nakagawa1 Received: 8 November 2018 / Accepted: 31 December 2018 © Japanese Association of Cardiovascular Intervention and Therapeutics 2019
Abstract Veno-arterial extracorporeal membrane oxygenation (VA ECMO) is a powerful device for treatment of patients with lifethreatening 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
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