Heyde Syndrome-Pathophysiology and Perioperative Implications

Abstract

Gastrointestinal (GI) bleeding in patients with calcific aortic valve stenosis (AVS), termed Heyde syndrome, was first described by Edward C. Heyde. The strong association between valvular replacement and the eradication of clinically significant GI bleeding confirmed an underlying pathophysiologic relationship. The rheologic stress created by AVS increases proteolysis of von Willebrand factor (VWF), resulting in loss of predominantly high-molecular-weight VWF (Hmw VWF). Angiodysplastic vessels present in patients with AVS, coupled with the lack of functioning Hmw VWF, increase the risk for GI bleeds. Aortic valve replacement, both surgical and transcatheter-based, is often a definitive treatment for GI bleeding, leading to recovery of Hmw VWF multimers. Perioperative management of patients involves monitoring their coagulation profiles with relevant laboratory tests and instituting appropriate management. Management can be directed in the following two ways: by improving internal release of VWF or by administration of external therapeutics containing VWF. It is important for perioperative physicians to obtain an understanding of the pathophysiology of this disease process and closely monitor the bleeding pattern so that targeted therapies can be initiated.

Keywords: Heyde syndrome; acquired Von Willebrand disease; aortic stenosis; perioperative; von Willebrand factor.