Heyde Syndrome Unveiled: A Case Report with Current Literature Review and Molecular Insights

Int J Mol Sci. 2024 Oct 14;25(20):11041. doi: 10.3390/ijms252011041.

Abstract

Heyde syndrome, marked by aortic stenosis, gastrointestinal bleeding from angiodysplasia, and acquired von Willebrand syndrome, is often underreported. Shear stress from a narrowed aortic valve degrades von Willebrand factor multimers, leading to angiodysplasia formation and von Willebrand factor deficiency. This case report aims to raise clinician awareness of Heyde syndrome, its complexity, and the need for a multidisciplinary approach. We present a 75-year-old man with aortic stenosis, gastrointestinal bleeding from angiodysplasia, and acquired von Willebrand syndrome type 2A. The patient was successfully treated with argon plasma coagulation and blood transfusions. He declined further treatment for aortic stenosis but was in good overall health with improved laboratory results during follow-up. Additionally, we provide a comprehensive review of the molecular mechanisms involved in the development of this syndrome, discuss current diagnostic and treatment approaches, and offer future perspectives for further research on this topic.

Keywords: Heyde syndrome; acquired von Willebrand deficiency; angiodysplasia; aortic stenosis; von Willebrand factor.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Angiodysplasia* / complications
  • Angiodysplasia* / diagnosis
  • Angiodysplasia* / therapy
  • Aortic Valve Stenosis* / complications
  • Aortic Valve Stenosis* / etiology
  • Gastrointestinal Hemorrhage* / etiology
  • Humans
  • Male
  • von Willebrand Diseases / complications
  • von Willebrand Diseases / diagnosis
  • von Willebrand Diseases / therapy
  • von Willebrand Factor / genetics
  • von Willebrand Factor / metabolism

Substances

  • von Willebrand Factor