We congratulate Hudzik and colleagues for their remarkable case of Heyde syndrome;1 however, our findings differ substantially.2 Our patient presented with relapsing gastrointestinal bleeding, angiodysplasia and severe calcific aortic stenosis and declined valve replacement. We started octreotide to reduce the incidence and severity of bleeding relapses and no new gastrointestinal bleeding was diagnosed over more than two years of follow-up. Currently, the patient has stable hemoglobin at 110 g/L, despite recent stenting of the right internal carotid artery, chronic renal failure, urothelial carcinoma of the bladder and adenocarcinoma of the neck.
We acknowledge the evidence pointing to Heyde syndrome as an acquired type IIA von Willebrand syndrome, caused by the loss of the largest polymers of von Willebrand factor (vWF) because of the high shear forces generated through the stenotic aortic valve.3 Work up in our patient failed to disclose any abnormalities of platelets, coagulation parameters or vWF levels and function, both at presentation and at several points during follow-up after the start of octreotide therapy. We did not perform vWF gel electrophoresis, the gold standard to show the loss of large vWF polymers.4 Despite this limitation, our findings seem to rule out an acquired type IIA von Willebrand syndrome.
We speculate type IIA von Willebrand syndrome could not provide the underlying mechanistic explanation for the association between aortic stenosis, angiodysplasia and gastrointestinal bleeding in some cases of Heyde syndrome. Additional hypotheses should be investigated in this subset of patients.