Heyde's syndrome: Acquired von Willebrand Syndrome in Severe Aortic Stenosis

Heyde’s syndrome: Acquired von Willebrand Syndrome in Severe Aortic Stenosis

Severe gastrointestinal bleeding can occur in those with severe aortic stenosis (Heyde’s syndrome). The initial description was in 1958 [1]. An important mechanism for the bleeding is deficiency of large von Willebrand factor multimers. This is due to the structural damage to the large protein when blood passes through the severely stenotic aortic valve as a high velocity jet. Loss of large multimers can be measured as the large von Willebrand factor multimer index and it is significant if it is less than 80% [2]. Anemia due to Heyde’s syndrome improves after aortic valve replacement. The gastrointestinal bleeding due to angiodysplasia though mostly described in severe aortic stenosis, has also been reported in hypertrophic obstructive cardiomyopathy [3].
Increased production of vascular endothelial growth factor (VEGF) due to reduced pulsatile flow and impaired intestinal flow is thought to be the reason for gastrointestinal angiodysplasis in aortic stenosis. For this reason thalidomide which inhibits the expression of VEGF has been used for treatment of Heyde’s syndrome [3]. Octreotide which can reduce intestinal blood loss by vasoconstriction in combination with thalidomide may be more beneficial for the initial treatment of Heyde’s syndrome [3].

Just as Heyde’s syndrome resolves after aortic valve replacement, it can resolve after transcatheter aortic valve implantation (TAVI) [4].

References

  1. Heyde E. Gastrointestinal bleeding in aortic stenosis. N Engl J Med. 1958;259:196
  2. Tamura T, Horiuchi H, Imai M, Tada T, Shiomi H, Kuroda M, Nishimura S, Takahashi Y, Yoshikawa Y, Tsujimura A, Amano M, Hayama Y, Imamura S, Onishi N, Tamaki Y, Enomoto S, Miyake M, Kondo H, Kaitani K, Izumi C, Kimura T, Nakagawa Y. 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(11):1115-23.
  3. Hvid-Jensen HS, Poulsen SH, Agnholt JS. Severe Gastrointestinal Bleeding in a Patient With Subvalvular Aortic Stenosis Treated With Thalidomide and Octreotide: Bridging to Transcoronary Ablation of Septal Hypertrophy. J Clin Med Res. 2015 Nov;7(11):907-10.
  4. Godino C, Lauretta L, Pavon AG, Mangieri A, Viani G, Chieffo A, Galaverna S, Latib A, Montorfano M, Cappelletti A, Maisano F, Alfieri O, Margonato A, Colombo A. Heyde’s Syndrome Incidence and Outcome in Patients Undergoing Transcatheter Aortic Valve Implantation. J Am Coll Cardiol. 2013 Feb 12;61(6):687-9.

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