Background: The Heyde syndrome consists of the association of gastrointestinal bleeding from angiodysplasia with aortic valve stenosis. Its existence has been repeatedly questioned or reconfirmed, and the proposed underlying mechanism is the degradation of a coagulation factor caused by the stenotic valve, which facilitates bleeding from angiodysplastic lesions.

Patient Case: We report the case of a patient with severe recurrent small-intestinal bleeding from angiodysplasia, diagnosed by a videocapsule, and aortic valve stenosis. He underwent aortic valve replacement with a bioprosthesis as an extreme life-saving procedure. The operation was followed by the cessation of bleeding for 10 months, then bleeding recurred, emergency bowel resection was needed, and was followed by a chain of events which led to the patient's death.

Conclusion: This case offers an extreme example of the challenging issues still involved in the management of patients with Heyde syndrome.

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http://dx.doi.org/10.1159/000098457DOI Listing

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Heyde's syndrome is a clinical entity that combines aortic stenosis, gastrointestinal angiodysplasia, and an acquired von Willebrand factor disorder. This syndrome is characterized by the association between aortic stenosis and recurrent gastrointestinal bleeding episodes, typically linked to angiodysplasias. Effective treatment requires addressing the underlying condition, specifically aortic stenosis, which leads to the structural destruction of coagulation proteins, resulting in the acquired von Willebrand factor disorder and perpetuating the bleeding.

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Heyde syndrome is a triad of aortic stenosis (AS), gastrointestinal (GI) bleeding from angiodysplasia, and acquired von Willebrand disease (vWD). It is hypothesized that stenotic aortic valves cleave von Willebrand factor (vWF) multimers, predisposing patients to bleeding from GI angiodysplasias. This hypothesis is supported by the observation that aortic valve replacement often leads to the resolution of GI bleeding.

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Importance: Heyde syndrome is the cooccurrence of aortic stenosis and gastrointestinal bleeding secondary to vascular lesions, including angiodysplasias. Several studies have demonstrated cessation of gastrointestinal bleeding after transcatheter aortic valve implantation (TAVI), but the etiology and effects on vascular lesions are largely unknown.

Objective: To examine the associations of TAVI with gastrointestinal vascular lesions and identify factors associated with recovery among patients with iron deficiency anemia and severe aortic stenosis.

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

Int J Mol Sci

October 2024

Center for Molecular Medicine and Stem Cell Research, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovica 69, 34000 Kragujevac, Serbia.

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.

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Article Synopsis
  • Heyde syndrome consists of anemia, aortic valve stenosis, and acquired von Willebrand syndrome, yet it remains under-recognized in clinical settings.
  • A 70-year-old woman with severe iron-deficiency anemia and aortic stenosis was diagnosed with Heyde syndrome after extensive testing that ruled out other bleeding sources.
  • Aortic valve replacement surgery improved her anemia and symptoms, highlighting the need for awareness of Heyde syndrome in cases of unexplained anemia, particularly when associated with aortic stenosis.
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