Gastrointestinal: Heyde's syndrome

Heyde's syndrome takes the name of Dr Edward Heyde, a physician in Vancouver, Canada. In a short letter to the New England Journal of Medicine in 1958, he indicated that he had seen ‘at least 10 patients with calcific aortic stenosis who had massive gastrointestinal bleeding for which we could discover no cause’. He thought that bleeding was probably related to ‘sclerotic vessels’ and this was confirmed as angiodysplasia in subsequent studies. Although some authors have questioned the association, other studies have documented improvement or cessation of bleeding after replacement of the diseased aortic valve. In 2002, another letter in the New England Journal of Medicine reported 2 patients with aortic stenosis and gastrointestinal bleeding who had an acquired form of von Willebrand disease (type IIA), perhaps because high sheer stress around the valve increased the breakdown of high molecular weight multimers of von Willebrand factor. These subtle abnormalities of coagulation resolved after replacement of the aortic valve. In Heyde's syndrome, angiodysplasia is usually located in the cecum or ascending colon. These sites appear to be similar to those of bleeding angiodysplasia without aortic stenosis. Angiodysplasia can be identified at colonoscopy in the majority of patients and can also be identified at angiography, particularly when there is significant bleeding. Therapeutic options depend on the clinical setting but include the use of argon plasma coagulation at colonoscopy and bowel resection, usually a right hemicolectomy. In the patient illustrated below, cecal angiodysplasia was identified at capsule endoscopy and at colonoscopy. The patient was a 64-year-old woman who was known to have aortic stenosis and was being considered for mechanical valve replacement. She was anemic and had positive fecal occult blood tests. Upper gastrointestinal endoscopy and colonoscopy at another hospital were reported as normal. She was referred to our center for video capsule endoscopy. The only abnormality was angiodysplasia in the cecum with a feeding vessel as shown in Figure 1. At repeat colonoscopy, abnormal blood vessels were identified in the cecum (Figure 2) but the feeding vessel was not visualized, perhaps because of the tamponade effect of insufflated air. The abnormal area was treated by argon plasma coagulation after which her hemoglobin returned to normal.

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