Acquired von willebrand syndrome associated with cardiovascular diseases

Hisanori Horiuchi, Tsuyoshi Doman, Koichi Kokame, Yoshikatsu Saiki, Masanori Matsumoto

Research output: Contribution to journalReview articlepeer-review

60 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)303-314
Number of pages12
JournalJournal of Atherosclerosis and Thrombosis
Issue number4
Publication statusPublished - 2019


  • Acquired von Willebrand’s syndrome
  • Aortic stenosis
  • ECMO
  • Heyde’s syndrome
  • Left ventricular assist device
  • Von Willebrand factor


Dive into the research topics of 'Acquired von willebrand syndrome associated with cardiovascular diseases'. Together they form a unique fingerprint.

Cite this