What is the management approach for patients with Heyde's syndrome, which involves aortic stenosis and gastrointestinal bleeding?

Heyde Syndrome Management

Patients with Heyde syndrome—the triad of severe aortic stenosis, gastrointestinal bleeding from angiodysplasia, and acquired von Willebrand disease—should undergo definitive aortic valve replacement (AVR) or transcatheter aortic valve implantation (TAVI) as the primary treatment, as correction of the aortic stenosis resolves the coagulopathy and typically stops recurrent gastrointestinal bleeding. 1, 2

Diagnostic Confirmation

Before proceeding with valve intervention, confirm all three components of Heyde syndrome:

• Severe aortic stenosis: Document by echocardiography with appropriate hemodynamic criteria 1
• Recurrent gastrointestinal bleeding: Establish history of repeated bleeding episodes requiring transfusion 3, 4
• Angiodysplasia: Identify vascular malformations via endoscopy (esophagogastroduodenoscopy, colonoscopy, or small bowel evaluation) 4
• Acquired von Willebrand disease: Demonstrate deficiency of high-molecular-weight multimers of von Willebrand factor by gel electrophoresis 1

The pathophysiology involves high shear stress from aortic stenosis causing proteolytic cleavage of large von Willebrand factor multimers, resulting in impaired primary hemostasis and bleeding from gastrointestinal angiodysplasias 1.

Initial Stabilization

Prior to definitive valve intervention:

• Transfuse packed red blood cells to correct anemia and maintain hemodynamic stability 3, 1
• Provide supportive care including bowel rest if active bleeding 1
• Optimize heart failure management with diuretics and afterload reduction as tolerated 2
• Avoid anticoagulation whenever possible to minimize bleeding risk 5

Definitive Treatment: Valve Intervention

Choice of Intervention

Proceed with aortic valve replacement (surgical AVR or TAVI) based on standard risk stratification for aortic stenosis. 6 The 2017 ACC Expert Consensus recommends multidisciplinary Heart Valve Team evaluation to determine optimal approach (TAVI vs. surgical AVR) based on surgical risk, anatomy, and patient factors 6.

• TAVI is appropriate for intermediate to high surgical risk patients with suitable anatomy 6, 3
• Surgical AVR is preferred for lower-risk patients, those requiring concomitant procedures, or when mechanical valve is indicated 6, 2

Prosthesis Selection

Use a bioprosthetic valve rather than mechanical prosthesis when possible to avoid lifelong anticoagulation that could exacerbate bleeding 5. However, mechanical valves can be used successfully if indicated by patient age and preferences, as valve replacement corrects the underlying coagulopathy 2.

The critical principle: Correction of aortic stenosis resolves the acquired von Willebrand disease and typically eliminates recurrent gastrointestinal bleeding, making valve replacement more important than intestinal resection. 5, 1

Post-Intervention Monitoring

Immediate Post-Procedural Period

• Monitor for recurrent bleeding during index hospitalization, as Heyde patients require transfusions more frequently (50% vs. 32% in non-Heyde GI bleeders) 3
• Assess for paravalvular leak after TAVI, as residual ≥mild paravalvular regurgitation is associated with higher rates of recurrent bleeding (73% vs. 38%) 3

Long-Term Follow-Up

• Surveillance for recurrent GI bleeding: Approximately 40% of Heyde patients experience recurrent bleeding within one year after TAVI, compared to 21% of non-Heyde GI bleeders 3
• Evaluate paravalvular leak if bleeding recurs, as this may require reintervention 3
• Monitor hemoglobin levels regularly to detect occult bleeding 4
• Repeat endoscopy if anemia or overt bleeding develops 4

Critical Pitfalls to Avoid

Do not perform intestinal resection before correcting aortic stenosis, as the coagulopathy will persist and bleeding will likely recur from other angiodysplasias 5. The bleeding originates from a systemic coagulopathy, not just localized vascular lesions.

Do not dismiss gastrointestinal bleeding as unrelated to cardiac disease in patients with aortic stenosis and systolic murmur—always consider Heyde syndrome in the differential diagnosis 1.

Do not overlook residual paravalvular leak after TAVI, as this may perpetuate the coagulopathy and allow continued bleeding 3. If significant paravalvular regurgitation persists with recurrent bleeding, consider reintervention.

Recognize that moderate to severe anemia (hemoglobin <11 g/dL) occurs in 52% of severe AS patients, and these patients have significantly higher rates of angiodysplasia (38% vs. 8%) and active bleeding (23% vs. 0%) throughout the entire gastrointestinal tract 4.