Understanding Heyde's Syndrome: The Association Between Aortic Stenosis and Angiodysplasia
The Pathophysiologic Link
Aortic stenosis causes gastrointestinal bleeding from angiodysplasia through acquired von Willebrand disease type IIA, where high shear stress across the stenotic valve destroys the large multimers of von Willebrand factor needed for hemostasis in high-flow vascular malformations. 1
The mechanism works as follows:
Severe aortic stenosis creates abnormally high shear stress that selectively destroys the high-molecular-weight (HMW) multimers of von Willebrand factor (VWF), producing an acquired coagulopathy equivalent to von Willebrand disease type IIA 2, 1
These large VWF multimers are specifically required to maintain hemostasis in high-flow conditions, such as those occurring in angiodysplastic arteriovenous malformations throughout the gastrointestinal tract 2
Angiodysplasia itself is a chronic degenerative condition that increases with age and shares cardiovascular risk factors with aortic stenosis, making both conditions commonly coexist in elderly patients 2
The bleeding occurs from previously latent angiodysplasia that becomes clinically apparent only when the acquired hematological defect develops 2
Management Strategy
Definitive Treatment: Aortic Valve Replacement
Aortic valve replacement—either surgical or transcatheter—is the definitive treatment that addresses both the hemodynamic problem and the bleeding diathesis, offering the best hope for long-term resolution. 2
Valve replacement should be strongly considered in most cases, particularly when the aortic stenosis is symptomatic, as it corrects the underlying coagulopathy rather than just treating the bleeding sites 2
Both surgical AVR and TAVI restore HMW multimers of VWF immediately and durably, with documented cessation of gastrointestinal bleeding following intervention 3
Angiodysplastic lesions may actually disappear after valve replacement, with endoscopic documentation showing complete resolution at 4 months and sustained absence at 2 years post-TAVI 3
Screening for iliofemoral peripheral arterial disease is recommended in all patients undergoing TAVI, as PAD is present in 20-30% of severe AS patients and increases procedural complications 4
Critical Valve Selection Consideration
If valve replacement is performed, a bioprosthetic valve is strongly preferred over a mechanical prosthesis to avoid the need for anticoagulation, which would exacerbate gastrointestinal bleeding from angiodysplasia. 5
Mechanical prostheses requiring anticoagulation can promote ongoing bleeding from angiodysplastic lesions, with documented cases requiring replacement with bioprosthetic valves to achieve hemostasis 5
The European guidelines recommend bioprosthetic valves for patients ≥65 years, which encompasses most patients with Heyde's syndrome 4
Temporizing Measures When Surgery Is Not Immediate
Endoscopic localization and cauterization of bleeding angiodysplasia can provide temporary relief but has high recurrence rates and should not delay definitive valve replacement. 2
Recurrent blood transfusions may offer symptomatic relief in patients deemed unfit for surgery when no discrete bleeding point can be identified 2
Aggressive blood pressure control is essential, with the American College of Cardiology recommending renin-angiotensin system blockers as preferred agents, while avoiding over-diuresis in patients with small LV cavities 6, 7
Diagnostic Workup in Suspected Heyde's Syndrome
Patients with severe aortic stenosis and moderate/severe anemia (hemoglobin <11 g/dL) have a 38% prevalence of gastrointestinal angiodysplasia and should undergo endoscopic evaluation. 8
Angiodysplasia can occur throughout the entire GI tract: stomach (43%), colon (48%), and small intestine (24%) in patients with severe AS and anemia 8
Active bleeding is significantly more common in AS patients with moderate/severe anemia (23.4%) compared to those without (0%) 8
Testing for HMW multimers of VWF can confirm the diagnosis of acquired von Willebrand disease type IIA, though this is not routinely necessary for clinical management 1, 3
Common Pitfalls to Avoid
Do not pursue repeated endoscopic interventions as primary therapy when definitive valve replacement is feasible, as this addresses only the symptom rather than the underlying cause 2
Avoid mechanical valve prostheses in patients with documented or suspected angiodysplasia, as mandatory anticoagulation perpetuates bleeding 5
Do not delay valve replacement in symptomatic patients due to concerns about anemia or bleeding risk, as the valve replacement itself resolves the coagulopathy 2
Recognize that both conditions increase with age and share cardiovascular risk factors, so their coexistence is common rather than coincidental 2