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Heyde Syndrome Resolution After AVR — EECC MCQ

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ModerateValvular Heart DiseaseHeyde Syndrome Resolution After AVREECC

A 65-year-old man with severe aortic stenosis presents with GI bleeding. His haematologist identifies acquired von Willebrand disease (loss of high-molecular-weight vWF multimers). After AVR, his vWF multimers normalise and GI bleeding resolves. This is an example of which syndrome?

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Correct answer: D — Heyde syndrome — the triad of calcific AS, acquired von Willebrand syndrome, and GI bleeding from angiodysplasia; AVR restores normal vWF multimer distribution and resolves bleeding

Heyde syndrome is increasingly recognised as a clinically significant entity. The high shear stress across the stenotic aortic valve causes proteolytic cleavage of large vWF multimers by ADAMTS13, producing acquired von Willebrand disease type 2A. These large multimers are essential for platelet adhesion at sites of angiodysplasia (which are common in the GI tract of elderly patients). The combination produces recurrent GI bleeding that may be refractory to endoscopic treatment. Crucially, AVR (surgical or TAVI) eliminates the shear stress, vWF multimers recover within days-weeks, and GI bleeding resolves — providing definitive proof of the causal relationship. Screening for acquired vWD should be considered in AS patients with unexplained anaemia or recurrent GI bleeding. TAVI has been specifically used for Heyde syndrome even when AS is not yet meeting conventional intervention criteria.

Reference: ESC/EACTS (2025): VHD Guidelines