This eponym is derived from E.C. Heyde, a general practitioner from Vancouver, Washington who observed in 1958 that patients with calcific aortic stenosis were prone to massive gastrointestinal bleeding.  This clinical observation now has a molecular insight (NEJM 2012; 367: 1954-56).

Submucosal angiodysplasia was identified as the source of GI bleeding.  This in turn was discovered to be related to an acquired von Willebrand’s syndrome.  What’s happening is that elevated shear stress coverts the globular von Willebrand polymer into an elongated asymmetric protein.  This conformational change exposes a site to the protease ADAMTS13 which binds  and cleaves the protein, leaving a less competent smaller von Willebrand factor.

Another observation is that the von Willebrand factor is essential for the role that platelets have in maintaining vascular integrity.  Degradation of von Willebrand factor as in Heyde’s syndrome allows for the development of the angiodysplasia in these patients and it leads to an intrinsic vascular diathesis in young patients with hereditary von Willebrand’s disease.

It is pretty cool to see how the science explains the clinical picture.