Acquired von Willebrand Syndrome and Chronic Anaemia: A Different Diagnostic Approach
  • Nuno Zarcos Palma
    Serviço de Medicina Interna, Centro Hospitalar Tâmega e Sousa, Penafiel, Portugal http://orcid.org/0000-0002-7036-9976
  • Mariana da Cruz
    Serviço de Medicina Interna, Centro Hospitalar Tâmega e Sousa, Penafiel, Portugal
  • Gisela Evaristo Vasconcelos
    Serviço de Medicina Interna, Centro Hospitalar Tâmega e Sousa, Penafiel, Portugal
  • Vitor Fagundes
    Serviço de Medicina Interna, Centro Hospitalar Tâmega e Sousa, Penafiel, Portugal
  • Lindora Pires
    Serviço de Medicina Interna, Centro Hospitalar Tâmega e Sousa, Penafiel, Portugal
  • Mari Mesquita
    Serviço de Medicina Interna, Centro Hospitalar Tâmega e Sousa, Penafiel, Portugal

Keywords

Heyde’s syndrome, hypertrophic obstructive cardiomyopathy, acquired von Willebrand syndrome, angiodysplasia, anemia

Abstract

Heyde’s syndrome is a form of acquired von Willebrand syndrome that consists of bleeding from intestinal angiodysplasia in the presence of aortic stenosis (AS). An association with obstructive hypertrophic cardiomyopathy, rather than AS, by a similar mechanism has also been described. We report the case of a 78-year-old woman with chronic anaemia and hypertrophic obstructive cardiomyopathy in whom intestinal angiodysplasia with active bleeding was identified by an unconventional method. In this case, the authors describe a different approach to reach the correct diagnosis.

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References

  • Heyde EC. Gastrointestinal bleeding in aortic stenosis. N Engl J Med 1958;259:196.

  • Loscalzo J. From clinical observation to mechanism — Heyde’s syndrome. N Engl J Med 2012;367;1954–1956.

  • Shimizu M, Masai H, Miwa Y. Occult gastrointestinal bleeding due to acquired von Willebrand syndrome in a patient with hyper-trophic obstructive cardiomyopathy. Intern Med 2007;46:481–485.

  • Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F, et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med 2003;349:343–349.

  • Maron BJ. Clinical course and management of hypertrophic cardiomyopathy. N Engl J Med 2018;379:655–668.

  • Warkentin TE, Moore JC, Morgan DG. Aortic stenosis and bleeding gastrointestinal angiodysplasia: is acquired von Willebrand’s disease the link? Lancet 1992;340:35–37.

  • Blackshear JL, Schaff HV, Ommen SR, et al. Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy. Mayo Clin Proc 2011;86:219.

  • Gill JC, Wilson AD, Endres-Brooks J, Montgomery RR. Loss of the largest von Willebrand factor multimers from the plasma of patients with congenital cardiac defects.Blood 1986;67:758.

  • Starke RD, Ferraro F, Paschalaki KE, et al. Endothelial von Willebrand factor regulates angiogenesis. Blood 2011;117:1071.

  • Tamura T, Horiuchi H, Imai M, et al. Unexpectedly high prevalence of acquired von Willebrand syndrome in patients with severe aortic stenosis as evaluated with a novel large multimer index. J Atheroscler Thromb 2015;22:1115.

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    Published: 2019-06-10
    Issue: Vol 6 No 6 (view)

    How to cite:
    1.
    Zarcos Palma N, da Cruz M, Vasconcelos GE, Fagundes V, Pires L, Mesquita M. Acquired von Willebrand Syndrome and Chronic Anaemia: A Different Diagnostic Approach. EJCRIM 2019;6 doi:10.12890/2019_001137.