Heyde's Syndrome - Pathophysiology

Pathophysiology

In the 45 years following its initial description, no plausible explanations could be found for the association between aortic valve stenosis and gastrointestinal bleeding. Indeed, the association itself was questioned by a number of researchers. A number of reports stressed, however, that replacement of the diseased aortic valve often led to resolution of the coagulopathy.

A 2003 study showed how the subtle form of von Willebrand disease present in Heyde syndrome patients resolved rapidly after aortic valve replacement of the stenosed aortic valve. The coagulation abnormality, the study poses, is possibly caused by the increased breakdown of the very large von Willebrand factor molecule by its natural catabolic enzyme (named ADAMTS13) under conditions of high shear stress around the valve.