Heyde's syndrome

Heyde's syndrome is a syndrome of aortic valve stenosis associated with gastrointestinal bleeding from colonic angiodysplasia. It is named after Dr. Edward C. Heyde, who first described the association in 1958.^[1] It is due to the induction of von Willebrand disease type IIA by the valvular stenosis.^[2]

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Signs and symptoms

Gastrointestinal hemorrage may present as hematemesis (vomiting blood), melena (tarry stool from altered blood) or hematochezia (fresh blood in stool). These symptoms usually lead to endoscopy of the upper and/or lower digestive tract (gastroscopy or colonoscopy). If this shows angiodysplasia, and the patient is known to have aortic stenosis, the combination is referred to as "Heyde's syndrome". It is not necessary for the aortic stenosis to lead to any other symptoms, but evidence of heart failure, syncope or chest pain may be present if the stenosis is severe.^[3]

Diagnosis

The tests normally performed for Von Willebrand disease are not necessarily positive, as the abnormality is subtle.^[4] Platelet function analysis (with ADP closure time) or von Willebrand factor electrophoresis identifies reduction of ULVWF (ultra-large von Willebrand factor).^[2]

Pathophysiology

In the 45 years following its initial description, no plausible explanations could be found for the association between aortic valve stenosis and gastrointestinal bleeding. Indeed, the association itself was questioned by a number of researchers.^[5] A number of reports stressed, however, that replacement of the diseased aortic valve often led to resolution of the coagulopathy.^[4][6]

A 2003 study showed how the subtle form of von Willebrand disease present in Heyde syndrome patients resolved rapidly after aortic valve replacement of the stenosed aortic valve. The coagulation abnormality, the study poses, is possibly caused by the increased breakdown of the very large von Willebrand factor molecule by its natural catabolic enzyme (named ADAMTS13) under conditions of high shear stress around the valve.^[2]

Therapy

Main article: Angiodysplasia

Symptomatic treatment can be given in the form of blood transfusions. Desmopressin (DDAVP) releases Factor VIII storage pools and is used in mild to moderate cases of von Willebrand disease.

Vincentelli et al argue that severe forms of Heyde's syndrome might be sufficient reason for aortic valve replacement, even if the stenosis is otherwise clinically unimportant and is not likely to cause complications.^[2]

Epidemiology

The exact prevalence of the syndrome is unknown. In a 2003 study it was found that of the 73 angiodysplasia patients who had also undergone echocardiograms, the prevalence of aortic stenosis was 31% (as opposed to 14% in the control group).^[7]

History

The syndrome was originally described in 1958 by Dr Edward C. Heyde, an internist living and working in Vancouver, WA in a letter to the New England Journal of Medicine. He reported ten patients with the association.^[1] A letter appearing shortly after confirmed an odds ratio of almost 3.0 between the two diseases.^[8]

References

1. ^ ^{a b} Heyde EC (1958). "Gastrointestinal bleeding in aortic stenosis". N. Engl. J. Med. 259: 196.
2. ^ ^{a b c d} Vincentelli A, Susen S, Le Tourneau T, et al (2003). "Acquired von Willebrand syndrome in aortic stenosis". N. Engl. J. Med. 349 (4): 343–9. doi:10.1056/NEJMoa022831. PMID 12878741.
3. ^ Warkentin TE, Moore JC, Anand SS, Lonn EM, Morgan DG (2003). "Gastrointestinal bleeding, angiodysplasia, cardiovascular disease, and acquired von Willebrand syndrome". Transfus Med Rev 17 (4): 272–86. PMID 14571395.
4. ^ ^{a b} Warkentin TE, Moore JC, Morgan DG (1992). "Aortic stenosis and bleeding gastrointestinal angiodysplasia: is acquired von Willebrand's disease the link?". Lancet 340 (8810): 35–7. doi:10.1016/0140-6736(92)92434-H. PMID 1351610.
5. ^ Gostout CJ (1995). "Angiodysplasia and aortic valve disease: let's close the book on this association". Gastrointest. Endosc. 42 (5): 491–3. doi:10.1016/S0016-5107(95)70058-7. PMID 8566646.
6. ^ Warkentin TE, Moore JC, Morgan DG (2002). "Gastrointestinal angiodysplasia and aortic stenosis". N. Engl. J. Med. 347 (11): 858–9. doi:10.1056/NEJM200209123471122. PMID 12226167.
7. ^ Batur P, Stewart WJ, Isaacson JH (2003). "Increased prevalence of aortic stenosis in patients with arteriovenous malformations of the gastrointestinal tract in Heyde syndrome". Arch. Intern. Med. 163 (15): 1821–4. doi:10.1001/archinte.163.15.1821. PMID 12912718.
8. ^ Goldman MJ (1958). "Aortic stenosis and gastrointestinal bleeding". N. Engl. J. Med. 259: 941.