Case Report
A surgical case of aortic stenosis with recurrent gastrointestinal bleeding: Heyde syndrome

https://doi.org/10.1016/j.ijscr.2018.10.078Get rights and content
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Highlights

  • We report successful surgical treatment of Heyde syndrome.

  • The incidence of Heyde syndrome is expected to increase in aging population.

  • Heyde syndrome should be considered in AS patients with gastrointestinal bleeding.

  • Radical surgery should be performed regardless of gastrointestinal bleeding.

Abstract

Introduction

The combination of aortic stenosis, acquired coagulopathy, and anemia due to gastrointestinal (GI) bleeding is described as Heyde syndrome.

Presentation of case

We report a surgical case of a 77-year-old man who was admitted because of melena and exertional chest compression. GI endoscopy could not reveal the origin of the GI bleeding. Conservative therapy including fasting and transfusion improved the anemia. Echocardiography demonstrated severe aortic stenosis (AS) with a hypertrophied left ventricle. Hematologic examination by gel electrophoresis showed deficiency of high-molecular-weight multimers of von Willebrand factor (vWF), resulting in the diagnosis of Heyde syndrome. He electively underwent aortic valve replacement (AVR) with a bioprosthesis using standard cardiopulmonary bypass. In the perioperative period, the patient had no recurrent anemia. He was discharged without subjective symptoms on postoperative day 18. The 20-month follow-up was unremarkable, with no episode of recurrent GI bleeding.

Discussion

Heyde syndrome is associated with acquired von Willebrand disease type IIA and AS. The true characteristic of von Willebrand disease type IIA is hemorrhagic diathesis caused by deficiency of high-molecular-weight multimers of vWF. Under the influence of high shear stress caused by AS, vWF is stretched and easily cleaved by vWF-cleaving protease. Consequently, it causes deficiency of high-molecular-weight multimers of vWF and primary hemostasis impairment. Therefore, the most effective treatment for Heyde syndrome is correction of AS. AVR can improve not only the hemodynamic status but also coagulopathy.

Conclusion

The differential diagnosis of patients with recurrent GI bleeding with concurrent systolic murmur should include Heyde syndrome.

Keywords

Case report
Aortic stenosis
Gastrointestinal bleeding
Heyde syndrome

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