Treatment of Heyde's Syndrome
The definitive treatment for Heyde's syndrome is aortic valve replacement, which resolves the gastrointestinal bleeding by correcting the acquired von Willebrand syndrome associated with severe aortic stenosis. 1, 2
Understanding Heyde's Syndrome
Heyde's syndrome is characterized by a triad of:
- Aortic stenosis
- Gastrointestinal bleeding from angiodysplasia
- Acquired von Willebrand syndrome (coagulopathy)
This condition typically affects elderly patients between 60-80 years of age and presents with recurrent episodes of gastrointestinal bleeding leading to iron-deficiency anemia requiring frequent blood transfusions.
Diagnostic Approach
To diagnose Heyde's syndrome, the following tests should be performed:
- Echocardiography to confirm severe aortic stenosis
- Endoscopic evaluation (upper endoscopy, colonoscopy, and capsule endoscopy) to identify angiodysplastic lesions
- Coagulation studies:
- Platelet function assays (closure times with collagen/ADP and collagen/epinephrine)
- von Willebrand factor (vWF) studies including vWF antigen, vWF:Ristocetin cofactor activity, and multimer analysis
Notably, standard coagulation studies may be normal despite the presence of acquired von Willebrand syndrome. Abnormal closure times may be the only laboratory evidence of the coagulopathy even when vWF activity appears normal. 3
Treatment Algorithm
1. Acute Management
- Blood transfusions for symptomatic anemia
- Intravenous fluid resuscitation for hemodynamic instability
- Temporary cessation of anticoagulants and antiplatelet medications if applicable
2. Definitive Treatment
- Aortic valve replacement (AVR) - surgical or transcatheter approach
- This addresses the root cause by eliminating the high shear stress across the stenotic valve that leads to degradation of von Willebrand factor multimers
- Resolution of bleeding typically occurs following successful valve replacement 2
3. Alternative Management (for non-surgical candidates)
- Endoscopic treatment of bleeding angiodysplastic lesions (argon plasma coagulation)
- Octreotide to reduce splanchnic blood flow
- Estrogen-progesterone therapy to improve vascular integrity
- Desmopressin to temporarily increase von Willebrand factor levels
Evidence for Treatment Efficacy
Multiple case reports demonstrate that aortic valve replacement leads to resolution of gastrointestinal bleeding in patients with Heyde's syndrome. In one case, an 80-year-old woman with severe aortic stenosis who required blood transfusions every 2 weeks experienced complete resolution of anemia following aortic valve replacement, with hemoglobin remaining stable at >10 mg/dL without further transfusions. 2
However, recurrence of bleeding can occur in some cases, particularly with bioprosthetic valves over time. One extreme case showed cessation of bleeding for 10 months following aortic valve replacement before recurrence, suggesting that ongoing monitoring is necessary. 4
Important Considerations and Pitfalls
- Delayed diagnosis: The connection between aortic stenosis and GI bleeding is often missed, leading to repeated endoscopic procedures without addressing the underlying cause
- Incomplete evaluation: Failure to perform capsule endoscopy may miss small bowel angiodysplasia when upper and lower endoscopies are negative
- Surgical risk assessment: Elderly patients with Heyde's syndrome often have multiple comorbidities that increase surgical risk
- Recurrence risk: Patients with bioprosthetic valves may experience recurrence of bleeding if valve degeneration occurs
Follow-up Recommendations
- Regular monitoring of hemoglobin levels
- Echocardiographic assessment of valve function
- Prompt evaluation of any recurrent gastrointestinal bleeding
By addressing the underlying aortic stenosis through valve replacement, both the hemodynamic abnormality and the associated coagulopathy can be corrected, leading to resolution of the gastrointestinal bleeding in most patients with Heyde's syndrome.