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Heyde Syndrome-Pathophysiology and Perioperative Implications.
Samhati Mondal1, Kimberly N Hollander1, Stephanie O Ibekwe2
1Department of Anesthesiology, Cardiac Anesthesia Division, University of Maryland School of Medicine, Baltimore, MD.
Heyde syndrome, characterized by gastrointestinal bleeding and aortic valve stenosis, is linked to a deficiency in high-molecular-weight von Willebrand factor. Valve replacement often resolves bleeding by restoring this crucial factor.
Area of Science:
- Cardiology
- Gastroenterology
- Hematology
Background:
- Heyde syndrome links calcific aortic valve stenosis (AVS) with gastrointestinal (GI) bleeding.
- This condition involves a deficiency in high-molecular-weight von Willebrand factor (Hmw VWF) due to rheologic stress from AVS.
- Angiodysplastic vessels in AVS patients exacerbate bleeding risk when Hmw VWF is compromised.
Purpose of the Study:
- To elucidate the pathophysiologic link between AVS and GI bleeding in Heyde syndrome.
- To highlight the role of von Willebrand factor (VWF) dysfunction in this condition.
- To outline perioperative management strategies for patients with Heyde syndrome.
Main Methods:
- Review of the pathophysiology connecting AVS, VWF proteolysis, and GI bleeding.
- Analysis of the impact of aortic valve replacement on GI bleeding and VWF recovery.
- Discussion of perioperative management including coagulation monitoring and therapeutic approaches.
Main Results:
- Aortic valve replacement (surgical or transcatheter) often definitively treats GI bleeding in Heyde syndrome.
- Valve replacement leads to the recovery of functional high-molecular-weight VWF multimers.
- Understanding VWF dynamics is key to managing GI bleeding in AVS patients.
Conclusions:
- Heyde syndrome pathophysiology involves AVS-induced VWF deficiency, increasing GI bleed risk.
- Aortic valve replacement is a primary treatment, restoring Hmw VWF and resolving bleeding.
- Targeted perioperative management, focusing on VWF status, is crucial for patient outcomes.
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