Heyde Syndrome — EECC MCQ

Correct answer: C — The triad of aortic stenosis, acquired von Willebrand syndrome (type 2A), and GI bleeding from angiodysplasia — high shear stress across the stenotic valve degrades large vWF multimers, causing a bleeding diathesis that resolves after AVR

Heyde syndrome is the association of: (1) calcific aortic stenosis; (2) acquired von Willebrand syndrome (type 2A) — high shear stress across the stenotic aortic valve causes proteolytic degradation of large vWF multimers by ADAMTS13, producing a qualitative vWF deficiency; (3) GI bleeding from angiodysplasia (arteriovenous malformations, particularly in the ascending colon). The degradation of large vWF multimers impairs platelet adhesion at sites of angiodysplasia. Crucially, AVR (surgical or TAVI) reverses the acquired vWF deficiency and eliminates the GI bleeding in most cases — demonstrating the causal relationship. This mechanism is analogous to the LVAD-associated acquired vWF syndrome and GI AVM bleeding. Screening for occult GI blood loss should be considered in AS patients with unexplained anaemia.

Reference: ESC/EACTS (2025): VHD Guidelines