Management of Heyde Syndrome
The definitive treatment for Heyde syndrome is aortic valve replacement (AVR), which addresses both the cardiac pathology and resolves gastrointestinal bleeding from angiodysplasia by restoring normal von Willebrand factor multimers. 1, 2
Understanding Heyde Syndrome
Heyde syndrome consists of three components that must be recognized:
- Aortic stenosis causing high shear stress across the narrowed valve 3
- Gastrointestinal bleeding from angiodysplasia, typically in the small intestine 4
- Acquired von Willebrand syndrome type 2A, where shear forces degrade high molecular weight von Willebrand factor multimers 3, 1
The pathophysiology is critical: the stenotic aortic valve creates mechanical shear stress that cleaves large von Willebrand factor multimers, leading to both bleeding tendency and angiodysplasia formation 3.
Diagnostic Workup
When Heyde syndrome is suspected (elderly patient with GI bleeding and cardiac murmur):
- Echocardiography to confirm and quantify aortic stenosis severity 4
- Endoscopic evaluation including esophagogastroduodenoscopy and colonoscopy to exclude common bleeding sources 4, 1
- Capsule endoscopy or double balloon endoscopy when initial endoscopy is negative, as angiodysplasias are often in the small intestine 1
- Platelet function assay and von Willebrand factor multimer analysis to document acquired von Willebrand syndrome 4, 1
Treatment Algorithm
Primary Treatment: Aortic Valve Replacement
Bioprosthetic valve replacement is preferred over mechanical valves to avoid the need for long-term anticoagulation, which would exacerbate bleeding risk 2. The evidence shows:
- GI bleeding resolves after AVR in most patients, with documented cessation of transfusion requirements 1, 2
- Improvement may take up to 12-20 months post-operatively as von Willebrand factor multimers normalize 1
- Both surgical AVR and transcatheter aortic valve replacement (TAVR) are effective 4
Bridging Strategies for Severe Bleeding
When bleeding is severe enough to preclude safe antiplatelet therapy needed for valve intervention:
- Argon plasma coagulation of identified angiodysplasias provides temporary hemostasis 3
- Blood transfusions and iron supplementation to maintain hemoglobin while planning definitive treatment 3, 4
- Systemic bevacizumab (antiangiogenic therapy) represents a novel approach for refractory, transfusion-dependent bleeding that cannot be controlled with endoscopic therapy, allowing stabilization before AVR 5
The bevacizumab approach is particularly important when patients are transfusion-dependent and cannot tolerate antiplatelet agents required for valve procedures 5.
Management Pitfalls to Avoid
- Do not perform intestinal resection before addressing the aortic stenosis - the bleeding will recur because the underlying coagulopathy persists 2
- Discontinue anticoagulants if the patient is on them, as they worsen bleeding without addressing the root cause 2
- Avoid mechanical valve prostheses that require lifelong anticoagulation in this bleeding-prone population 2
- Do not assume bleeding has stopped immediately after AVR - monitor for 12-20 months as normalization of hemostasis is gradual 1
When AVR is Declined or Contraindicated
If the patient refuses or cannot undergo valve replacement 3:
- Continue endoscopic ablation of angiodysplasias with argon plasma coagulation as needed 3
- Maintain hemoglobin with transfusions and iron supplementation 3
- Consider systemic bevacizumab for refractory cases, though long-term data are limited 5
- Close monitoring with serial hemoglobin checks and endoscopy as clinically indicated 3
However, this conservative approach does not address the underlying pathophysiology and patients remain at risk for recurrent bleeding.