A Vicious Circle: Heyde Syndrome in Mild Aortic Stenosis

Abstract

Patients with severe aortic stenosis have an increased risk of gastrointestinal (GI) bleeding that can be due to angiodysplasias in the GI tract; this association has been termed Heyde syndrome. High gradients across a severely stenotic aortic valve cause disruption of the von Willebrand factor and deficiency of large von Willebrand multimers. In one study, nine out of 42 (21%) patients with severe aortic stenosis had a history of bleeding, most often from skin or mucosal sites. In the same study, two out of eight patients with moderate aortic stenosis also had a history of hemorrhagic syndrome that required treatment. True mild aortic stenosis has, at least to our knowledge, not been described in the literature as a cause for Heyde syndrome. We here present a case of a 42-year-old man with a body weight of 160 kg who was diagnosed with Heyde syndrome in mild aortic stenosis. Loss of large von Willebrand multimers were proven by multimeric analysis. Gradients across a mildly stenotic aortic valve were significantly increased secondary to high-output cardiac state, which was itself caused by the combination of anemia and high body weight. Once the anemia was corrected and the patient lost 20% of his body weight (30 kg) with diuresis, the high-output state and flow across the aortic valve improved, gradients decreased, and the patient had no further episode of GI bleeding. We conclude that the patient was in a vicious circle where anemia eventually triggered further GI blood loss caused by Heyde syndrome in high-output cardiac state.