What is Heyde’s syndrome?
That is Heyde’s syndrome?
Hao_Tran (Hao Tran) #1
plin (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?)
- Tight aortic stenosis uncoils the larger molecular weight vWF rendering it vulnerable to cleavage by ADAMTS13. Faulty vWF leads to faulty platelets.
- 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.