A Case of Heyde's Syndrome With Subvalvular Aortic Stenosis

Heyde's syndrome is a constellation of severe aortic stenosis, gastrointestinal arteriovenous malformations (AVMs), and an acquired von Willebrand type 2A coagulopathy resulting in moderate-to-severe gastrointestinal bleeding. Additional cardiac lesions have been observed to cause Heyde's syndrome including aortic regurgitation, mitral regurgitation, aortic/mitral valve prosthetic dysfunction, ventricular septal defects, hypertrophic cardiomyopathy, left ventricular assist devices, and extracorporeal life support devices. Repairing the cardiac lesion or removing the device decreases the incidence of gastrointestinal bleeding by normalizing the acquired von Willebrand coagulopathy and decreasing the amount of gastrointestinal AVMs likely to bleed. We describe a case of a 67-year-old woman found to have Heyde's syndrome arising from a subvalvular aortic membrane resulting in severe subaortic stenosis with no other significant cardiac lesion. She underwent successful resection of the membrane with septal myectomy, relieving the severe subaortic stenosis and resolving her anemia. Years later, she re-presented with severe gastrointestinal bleeding from gastrointestinal malformations. Early recognition of these cardiac lesions with gastrointestinal bleeds may help decrease the morbidity and mortality that Heyde's syndrome portends and provide evidence for early intervention.