What is the management approach for Heyde syndrome?

Management Approach for Heyde Syndrome

Aortic valve replacement is the definitive treatment for Heyde syndrome as it addresses both the aortic stenosis and resolves the gastrointestinal bleeding by correcting the acquired von Willebrand syndrome.

What is Heyde Syndrome?

Heyde syndrome is characterized by the clinical triad of:

• Severe aortic stenosis
• Gastrointestinal bleeding from angiodysplasia
• Acquired von Willebrand syndrome type IIA

Pathophysiology

The pathophysiological mechanism involves:

• High shear stress across the stenotic aortic valve causes stretching of von Willebrand factor (vWF)
• This stretched vWF is more easily cleaved by vWF-cleaving protease
• Results in deficiency of high-molecular-weight multimers of vWF
• Leads to impaired primary hemostasis and bleeding from pre-existing gastrointestinal angiodysplasias

Diagnostic Approach

1. Cardiovascular evaluation:

   • Echocardiography to confirm and quantify aortic stenosis
   • Assessment of left ventricular function

2. Gastrointestinal evaluation:

   • Endoscopic studies (esophagogastroduodenoscopy, colonoscopy)
   • If negative, consider capsule endoscopy or mesenteric angiography to identify angiodysplasias

3. Hematologic evaluation:

   • Complete blood count to assess anemia
   • Coagulation profile
   • vWF antigen levels and multimer analysis to detect deficiency of high-molecular-weight multimers

Management Algorithm

Step 1: Initial Stabilization

• Blood transfusions for significant anemia
• Manage acute gastrointestinal bleeding episodes

Step 2: Definitive Treatment

• Aortic valve replacement (AVR) - surgical or transcatheter approach 1, 2, 3
  • Bioprosthetic valves are preferred over mechanical valves to avoid the need for long-term anticoagulation 1
  • Transcatheter aortic valve implantation (TAVI) is an option for high-risk surgical patients 4

Step 3: Post-AVR Monitoring

• Monitor for recurrent bleeding
• Follow hemoglobin levels
• Note that gastrointestinal bleeding may take up to 2 weeks to resolve completely after AVR 2

Important Considerations

• Timing of intervention: AVR should be performed before considering intestinal resection for angiodysplasias 1
• Anticoagulation management: Minimize or avoid anticoagulants when possible to reduce bleeding risk 1
• Residual paravalvular leakage: Patients with residual paravalvular leakage after TAVI may have higher rates of recurrent gastrointestinal bleeding (73.3% vs 38.1%) 4

Outcomes

• Resolution of gastrointestinal bleeding occurs in most patients following AVR
• In one study, patients had stable hemoglobin levels at 2-month and 6-month follow-up with no further episodes of gastrointestinal bleeding 2
• Another case reported no recurrent anemia or gastrointestinal bleeding at 20-month follow-up after AVR 3

Pitfalls to Avoid

1. Delayed diagnosis: Consider Heyde syndrome in elderly patients with recurrent gastrointestinal bleeding and a systolic murmur 3, 5

2. Incomplete evaluation: Ensure thorough gastrointestinal evaluation, as bleeding sources may be difficult to identify

3. Inappropriate treatment sequencing: Avoid intestinal resection before addressing the aortic stenosis 1

4. Overlooking residual valve issues: Patients with residual paravalvular leakage after TAVI have higher rates of recurrent bleeding 4