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Iron deficiency anaemia and calcific aortic stenosis are common findings in elderly patients. Heyde's syndrome, the association of aortic stenosis and gastrointestinal bleeding causing recurrent iron deficiency anaemia, is rare.
In October 2002, a woman aged 68 became dyspnoeic with decreased exercise tolerance and episodic chest pain. Prompted by her 8-year-old granddaughter, who on hugging her had commented that her heart 'sounded funny', she consulted her general practitioner, who diagnosed aortic stenosis, later confirmed by echocardiogram. For 2 years she had been under investigation for recurrent iron deficiency anaemia, which had remained unexplained despite upper gastrointestinal endoscopy, duodenal biopsy and colonoscopy. Additionally, during the previous 4 years she had experienced intermittent epistaxis. In December 2002, she had several episodes of melaena and her anaemia worsened, necessitating repeated blood transfusion. Heyde's syndrome was considered, and enteroscopy revealed bleeding jejunal angiodysplasia. The bleeding areas were coagulated by argon beam diathermy and the patient was placed on combined hormone replacement therapy. Seven weeks later, no jejunal lesions were visible on capsule endoscopy. The patient underwent aortic valve replacement and nine months later she had experienced no further episodes of bleeding.
Heyde's syndrome is named after Dr E C Heyde who in 1958 described ten cases of aortic stenosis and massive gastrointestinal bleeding for which he could discover no cause.1 The prevalence of the condition is unknown. In one retrospective analysis, 2.6% of patients with aortic stenosis, compared with 0.025% of a control group, had idiopathic gastrointestinal bleeding.2 Subsequent reports have implicated gastrointestinal angiodysplasia as a possible source of the bleeding but the precise relation between aortic stenosis and angiodysplasia is unclear. A causal relation is possible, but another possibility is that they coexist as age-related phenomena.
The blood vessel dilatation in gastrointestinal angiodysplasia is thought to arise from long-term obstruction within the submucosal mesenteric venous system, leading to loss of precapillary sphincters and formation of arteriovenous communications. A possible mechanism for the bleeding in Heyde's syndrome is an acquired form of von Willebrand's disease (type IIa) due to the aortic stenosis. High shear rates across the stenotic valve lead to increased consumption of high-molecular-weight multimers of von Willebrand factor (vWF). The subsequent functional deficit of vWF predisposes to bleeding. In a recent study, 21% of patients with severe aortic stenosis experienced skin or mucosal bleeding. Most of the patients had vWF abnormalities, the severity of which related to the degree of stenosis.3 Gastrointestinal angiodysplasia may be diagnosed by upper gastrointestinal endoscopy, enteroscopy, colonoscopy or capsule endoscopy. The lesions are found most commonly in the small bowel.
For bleeding angiodysplasia, one treatment option is combined oestrogen and progesterone therapy, which can reduce transfusion requirements by up to half.4 However, the possible benefits of hormone therapy should be set against cardiovascular risks. An alternative is to treat bleeding angiodysplasia at endoscopy with argon beam diathermy, but it is unknown whether this affects the natural history of the disease and risk of subsequent bleeding. Surgical options are bowel resection or aortic valve replacement. There have been several case reports of cessation of bleeding following aortic valve replacement.5 In a retrospective study of 91 patients with aortic stenosis and unexplained gastrointestinal bleeding King et al. found that bleeding ceased in 93% of patients treated by valve replacement compared with 5% of those managed by laparotomy with or without bowel resection.6
FH and CSO'B are undergraduate students at the University of Bristol and University College London and this paper forms part of a special study module by FH.