What is the treatment for Heyde's syndrome, a condition characterized by aortic stenosis and gastrointestinal (GI) bleeding?

From the Guidelines

The primary treatment for Heyde's syndrome is aortic valve replacement (AVR), which addresses the underlying aortic stenosis and typically resolves the gastrointestinal bleeding. Surgical AVR is the gold standard, though transcatheter aortic valve replacement (TAVR) may be considered for high-risk surgical patients 1. Before valve replacement, management focuses on supportive care including:

• Blood transfusions to address anemia
• Iron supplementation (oral or IV iron depending on severity)
• Temporary cessation of anticoagulants and antiplatelets if possible Endoscopic treatment of bleeding gastrointestinal arteriovenous malformations (AVMs) may be performed using argon plasma coagulation or electrocoagulation, but these interventions typically provide only temporary relief as new lesions often develop. The effectiveness of AVR stems from its correction of the acquired von Willebrand factor deficiency that develops in Heyde's syndrome, as supported by the guidelines for the management of patients with valvular heart disease 1. The high shear stress across the stenotic aortic valve causes degradation of von Willebrand factor multimers, leading to platelet dysfunction and increased bleeding risk from pre-existing GI AVMs. After valve replacement, von Willebrand factor levels typically normalize within days to weeks, resolving the bleeding tendency in approximately 80-90% of patients. Multimodality imaging strategies, including Doppler echocardiography, are essential for assessing the severity of aortic stenosis and guiding therapeutic decision-making 1. However, the primary goal of treatment in Heyde's syndrome remains the correction of the underlying aortic stenosis through AVR, which has been consistently shown to improve morbidity, mortality, and quality of life in affected patients.

From the Research

Treatment Options for Heyde's Syndrome

The treatment for Heyde's syndrome, a condition characterized by aortic stenosis and gastrointestinal (GI) bleeding, involves several approaches. The key treatments include:

• Aortic valve replacement (AVR) to correct aortic stenosis, which can improve hemodynamic status and coagulopathy 2, 3, 4
• Systemic bevacizumab, a monoclonal antibody directed against vascular endothelial growth factor, as a salvage therapy for persistent severe bleeding and anemia in Heyde syndrome following AVR 5, 6
• Hematologic support with red cell transfusion and intravenous iron to manage bleeding and anemia 5, 6

Rationale Behind Treatment Options

The rationale behind these treatment options is based on the pathophysiology of Heyde syndrome, which involves:

• The rheologic stress created by aortic stenosis increasing proteolysis of von Willebrand factor (VWF), resulting in loss of high-molecular-weight VWF multimers 3, 4
• The association between valvular replacement and the eradication of clinically significant GI bleeding, confirming an underlying pathophysiologic relationship 4
• The potential for systemic antiangiogenic therapy to target dysregulated angiogenesis in Heyde syndrome 5, 6

Key Considerations

Key considerations in the treatment of Heyde syndrome include:

• Monitoring coagulation profiles with relevant laboratory tests and instituting appropriate management 4
• Improving internal release of VWF or administering external therapeutics containing VWF 4
• The potential for persistent bleeding and anemia after AVR, and the need for additional therapies such as systemic bevacizumab 5, 6