Heyde Syndrome: Aortic Stenosis and Beyond

Heyde described aortic stenosis and gastrointestinal bleeding in the 1950s. Since then, a link with intestinal angiodysplasia and abnormalities of von Willebrand factor (VWF) has been noted. Loss of the highest-molecular-weight multimers of VWF and bleeding also have been described in subaortic stenosis in hypertrophic cardiomyopathy, in isolated mitral and aortic insufficiency, in endocarditis, in patients with prosthetic valve stenosis or regurgitation, and in patients with left ventricular assist devices (LVADs). Bleeding tends to recur with local treatment of angiodysplasias, whereas cardiac repair or removal of LVAD eliminates VWF dysfunction is curative of bleeding in the majority.