Heyde syndrome

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Definition

Heyde syndrome refers to a triad of aortic stenosis, an acquired coagulopathy (von Willebrand syndrome type 2A) and anaemia due to bleeding from intestinal angiodysplasia, or from an idiopathic site.

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Heyde syndrome is

  • aortic stenosis
  • causing an acquired von Willebrand syndrome type 2A due to shear stress
  • causing an anaemia due to bleeding from gastrointestinal angiodysplasia

Pathogenesis

The relative high velocity of blood flow within telangiectasia requires the largest multimers of von Willebrand factor (vWF) to maintain haemostasis. This could account for haemorrhage first manifesting in these lesions.[1]

Evidence

A controlled prospective study of 288 consecutive patients with aortic stenosis found a higher incidence of prolonged bleeding time in the patients with aortic stenosis, and the bleeding time fell to within normal limits following surgery. [2] In a study of 3 cohorts of patients enrolled at endoscopy, in which 9 had diverticular haemorrhage, 9 had non-bleeding gastrointestinal angiodysplasia and 9 had bleeding gastrointestinal angiodysplasia, it was found that 8 of the patients in the last group had loss of large multimers of vWF, and 7 of these patients had aortic stenosis, ranging from mild (1.5cm2) to severe (0.5cm2). The vWF profiles of all the other patients were normal. [3] In a study of 50 consecutive patients with aortic stenosis, 84% of whom were treated with aortic valve replacement, 21% of patients with severe aortic stenosis suffered from cutaneous or mucosal bleeding, and 67-92% had haematological abnormalities which correlated with the severity. Haematological investigations were performed 1 day, 7 days, and 6 months post-operatively. The abnormalities corrected on the first day, but tended to recur at six months, especially when there was a mismatch between patient and prosthesis.[4] In another study of 29 patients undergoing surgery for severe aortic stenosis, Yoshida and colleagues have shown an electrophoretic deficit of large multimers of vWF one month preoperatively. 8 of the 29 had symptomatic bleeding in the 6 months prior to surgery, all without anticoagulants. One month after surgery, the deficit had resolved, in all but the patients who had aortic valve prosthesis mismatch. 50 consecutive patients with severe mitral regurgitation were studied at the same time, but no differences in pre- and postoperative vWF were found. [5]

Investigation and treatment

In von Willebrand syndrome type 2A, routine screening tests for von Willebrand syndrome are usually normal. The gold standard is gel electrophoresis of vWF. [6] Patients with Heyde syndrome who are treated by intestinal resection generally continue to bleed from other sites, while aortic valve replacement usually cures the clotting disorder and anaemia. Treatments of von Willebrand syndrome type 2A itself include factor VIII/vWF concentrates and desmopressin, but acquired von Willebrand syndrome does not respond well to these. [7] Combined oestrogen and progesterone have been used to reduce bleeding from angiodysplasia, although the mechanism of action is not understood. [8]

References