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

Tamura, Toshihiro; Horiuchi, Hisanori; Obayashi, Yuki; Fuki, Masayuki; Imanaka, Miyako; Kuroda, Maiko; Nishimura, Shunsuke; Amano, Masashi; Sakamoto, Jiro; Tamaki, Yodo; Enomoto, Soichiro; Miyake, Makoto; Kondo, Hirokazu; Izumi, Chisato; Nakagawa, Yoshihisa

doi:10.1007/s12928-019-00568-y

Cited by 21 publications

(27 citation statements)

References 12 publications

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“…It is cleaved by ADAMTS-13, glycosylated and cleared from the bloodstream with a half-life of 12-20 h. 25 The mechanical forces of aortic stenosis, left ventricular assist device, and extracorporeal membrane oxygenation can easily shear and deform the high molecular weight multimers into smaller fragments, which impairs thrombosis and interferes with platelet aggregation. 26,27 von Willebrand factor fragmentation may, by itself, be pro-angiogenic and potentially involved in GI hemorrhage in these severe settings. 26,28 Cross-sectional imaging Cross-sectional imaging techniques for evaluation of the small bowel include helical computed tomography enterography/enteroclysis (CTE), helical CT angiography, and magnetic resonance enterography/enteroclysis (MRE).…”

Section: --mentioning

confidence: 99%

“…It is cleaved by ADAMTS‐13, glycosylated and cleared from the bloodstream with a half‐life of 12–20 h . The mechanical forces of aortic stenosis, left ventricular assist device, and extracorporeal membrane oxygenation can easily shear and deform the high molecular weight multimers into smaller fragments, which impairs thrombosis and interferes with platelet aggregation . von Willebrand factor fragmentation may, by itself, be pro‐angiogenic and potentially involved in GI hemorrhage in these severe settings …”

Section: Diagnosismentioning

confidence: 99%

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Management of obscure gastrointestinal bleeding: Comparison of guidelines between Japan and other countries

Ohmiya

2019

Digestive Endoscopy

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Small‐bowel bleeding accounts for the majority of obscure gastrointestinal bleeding, but it is caused by various types of small bowel disease, upper gastrointestinal disease, and colorectal disease. For the diagnosis, history taking and physical examination are required, leading to a determination of what diseases are involved. Next, cross‐sectional imaging, such as computed tomography, should be carried out, followed by the latest enteroscopy such as small bowel capsule endoscopy and deep enteroscopy according to the severity of hemorrhage and patient condition. After a comprehensive diagnosis, medical, enteroscopic, or surgical treatment should be selected. This article reviews recent advances in the endoscopic diagnosis of obscure gastrointestinal bleeding and compares perspectives of the management of obscure gastrointestinal bleeding in Japan with that in other countries.

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Section: --mentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

Management of obscure gastrointestinal bleeding: Comparison of guidelines between Japan and other countries

Ohmiya

2019

Digestive Endoscopy

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“…It is estimated that ECMO extracorporeal blood undergoes high shear stress, leading to the uncoiling of von Willebrand factor resulting in a state of thrombosis, fibrinolysis, and impaired platelet function [3]. Several studies have reported acquired von Willebrand syndrome in patients undergoing cardiopulmonary bypass and VA/VV ECMO [4,5].…”

Section: Discussionmentioning

confidence: 99%

Acquired factor XIII deficiency in two patients with bleeding events during veno-venous extracorporeal membrane oxygenation treatment

et al. 2019

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We report two cases of acquired factor XIII deficiency with bleeding events during veno-venous extracorporeal membrane oxygenation (ECMO). Case 1: A 76-year-old man diagnosed with aspiration pneumonia after near-drowning was started on ECMO. Later, the patient presented with hemoptysis and anemia. Blood tests showed a decreased factor XIII activity of 29%. Although the patient recovered after receiving 1200 International Units of factor XIII concentrate, the patient had another episode of decreased factor XIII activity and bloody stool and was treated again with factor XIII concentrate. Case 2: A 48-year-old female diagnosed with pneumonia was started on ECMO. Soon after, she presented with hemoptysis and anemia. Blood tests showed a decreased factor XIII activity of 39%. The patient was treated with 720 IU of factor XIII concentrate with good recovery. Acquired factor XIII deficiency cannot be detected by routine coagulation tests, therefore it may be under-diagnosed in the ICU. Detection of acquired factor XIII deficiency is essential when treating a bleeding ECMO patient.

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“…Percutaneous cardiopulmonary support (PCPS), otherwise known as V-A ECMO or extracorporeal life support (ECLS), is used for the treatment of acute circulatory failure. PCPS is also associated with bleeding and AVWS is noted as one of its major cause 85, 86) . AVWS caused by PCPS is far more severe, with much lower VWF large multimer indces, compared to that caused by aortic stenosis 86) .…”

Section: Avws Caused By Mechanical Circulatory Supportmentioning

confidence: 99%

Acquired von Willebrand Syndrome Associated with Cardiovascular Diseases

Horiuchi

Doman

Kokame

et al. 2019

JAT

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The blood glycoprotein von Willebrand factor (VWF) plays an important role in hemostasis and thrombosis. VWF is produced and secreted as large multimers by endothelial cells and megakaryocytes. It is then cleaved in a sheer-stress dependent manner by a specific protease, ADAMTS13, into multimers consisting of 2–80 subunits. Among VWF multimers, high molecular weight (HMW) multimers play important roles in platelet aggregation. Therefore, their loss induces a hemostatic disorder known as von Willebrand disease (VWD) type 2A. Various cardiovascular diseases, such as aortic stenosis, hypertrophic obstructive cardiomyopathy (HOCM), and several congenital structural diseases, as well as mechanical circulatory support systems, generate excessive high shear stress in the bloodstream. These cause excessive cleavage of VWF multimers resulting in a loss of HMW multimers, known as acquired von Willebrand syndrome (AVWS), a hemostatic disorder similar to VWD type 2A. Bleeding often occurs in the gastrointestinal tract since a fragile angiodysplasia develops associated with these diseases. Radical treatment for AVWS is to remove the pathological high shear causing AVWS.

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

Cited by 21 publications

References 12 publications

Management of obscure gastrointestinal bleeding: Comparison of guidelines between Japan and other countries

Management of obscure gastrointestinal bleeding: Comparison of guidelines between Japan and other countries

Acquired factor XIII deficiency in two patients with bleeding events during veno-venous extracorporeal membrane oxygenation treatment

Acquired von Willebrand Syndrome Associated with Cardiovascular Diseases

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