Acquired von Willebrand syndrome due to cardiac disorders was initially described in aortic stenosis and congenital heart defects. However, case series over several years have reported gastrointestinal bleeding due to angiodysplasia in hypertrophic cardiomyopathy (HCM). The association of gradient to severity of von Willebrand factor dysfunction in aortic stenosis was first defined in 2003 and in hypertrophic cardiomyopathy in 2008. Subsequent studies have further confirmed that platelet function analyzer 100 (PFA) and quantitative VWF multimers are strongly associated with gradients, that they function as biomarkers in demonstrating treatment responses, that bleeding is as prevalent as in aortic stenosis, and that septal reduction therapy can be curative of transfusion-dependent gastrointestinal hemorrhage from angiodysplasia.