Heyde Syndrome: Aortic Stenosis and Beyond

Area of Science:

• Cardiology
• Gastroenterology
• Hematology

Background:

• Aortic stenosis has been associated with gastrointestinal bleeding since the 1950s.
• A link between intestinal angiodysplasia and von Willebrand factor (VWF) abnormalities has been established.
• VWF dysfunction, specifically loss of highest-molecular-weight multimers, is observed in various cardiac conditions.

Purpose of the Study:

• To explore the relationship between cardiac conditions, VWF abnormalities, and gastrointestinal bleeding.
• To evaluate the efficacy of different treatment strategies for bleeding associated with VWF dysfunction.

Main Methods:

• Review of existing literature and case studies linking cardiac conditions with VWF abnormalities and bleeding.
• Analysis of treatment outcomes for gastrointestinal bleeding in patients with underlying cardiac issues.

Main Results:

• Loss of highest-molecular-weight VWF multimers is a common finding in patients with aortic stenosis, hypertrophic cardiomyopathy, valvular insufficiency, endocarditis, prosthetic valves, and left ventricular assist devices (LVADs).
• Gastrointestinal bleeding recurs frequently when angiodysplasias are treated locally.
• Cardiac repair or LVAD removal effectively resolves VWF dysfunction and cures bleeding in most cases.

Conclusions:

• Vascular abnormalities and associated bleeding in aortic stenosis are often linked to acquired von Willebrand factor dysfunction.
• Addressing the underlying cardiac condition (e.g., valve repair, LVAD removal) is curative for bleeding related to VWF dysfunction.
• Local treatment of angiodysplasias is less effective for bleeding associated with these cardiac conditions.