That is Heyde’s syndrome?

That is Heyde’s syndrome?
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(Hao Tran) #1

What is Heyde’s syndrome?


(Paul Lin) #2

Heyde syndrome is a triad of

  • aortic stenosis (examination/echo)
  • faulty platelet function (high-molecular-weight vWF multimer levels reduced)
  • anaemia due submucosal angiodysplasia (gas, colonoscopy, pillcam, enteroscopy; missed?)

Mechanism

  • Tight aortic stenosis uncoils the larger molecular weight vWF rendering it vulnerable to cleavage by ADAMTS13. Faulty vWF leads to faulty platelets.

Approach

  • DDx - Suspect Heyde’s in patients with GI bleeding without a cause and signs of aortic stenosis (a common association that Dr E.C. Heyde, a GP working in the Vancouver Clinic in Vancouver, Washington noted in 1958)
  • Ix - levels of vWF may be normal on screening but portion of high-molecular weight multimers are low, e.g. 2% rather than the usual 20%
  • Rx - resolution of the anaemia usually follows AV replacement or TAVI. Intestinal resection is ineffective as patients bleed from other sites.


http://www.nejm.org.acs.hcn.com.au/doi/full/10.1056/NEJMcibr1205363?query=recirc_curatedRelated_article