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Heyde's syndrome

Heyde's syndrome is a syndrome of aortic valve stenosisassociated with gastrointestinal bleedingfrom colonicangiodysplasia. It is namedafter Dr. Edward C. Heyde, who first described the association in 1958.

Inhaltsverzeichnis

1 Signs and symptoms
2 Diagnosis
3 Pathophysiology
4 Therapy
5 History
6 References
7 See also

Signs and symptoms

Aortic valve stenosisis a cause of heart failureand several other cardiac problems. In most patients, however, it goes unsuspected. A number of patients develops a bleedingtendency resembling that of platelet-derived coagulationproblems, e.g. nosebleeds, bruising. Some bleed from angiodysplasias(small vascular malformations) of the colon. This can be subtle, causing mild anemia, or serious, warranting blood transfusionsand surgeryto control bleeding.

Diagnosis

Angiodysplasiasare usually identified on colonoscopy. In some patients, the cause of bleeding cannot be identified on colonoscopy, and only angiographyof the colonic vasculature can identify the location of the angiodysplasias.

Aortic valve stenosisis identified on echocardiography(ultrasoundstudies of the heart), where a decreased Orifice Area of the aortic valveimplies stenosis.

Routine testing for Von Willebrand disease(see below) generally fails to identify the coagulationabnormality, as only the high-molecular weight-multimers (HMWM) of von Willebrand factorare decreased. Only dynamic plateletfunction analysis (PFA) might be of some use in this respect.

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. A number of reports stressed, however, that replacement of the diseased aortic valve often led to resolution of the coagulopathy.

Warkentin et al (1992) proposed that the link between aortic stenosis and bleeding was due to a mild form of von Willebrand disease. Some later reports confirmed the suspicion, including a 2002 study by the same group that showed resolution of the coagulation abnormality after aortic valve replacement.

In 2003a study by Vincentelli et al finally gave an explanation for the remarkable phenomenon. It showed how the subtle form of von Willebrand diseasepresent in Heyde syndrome patients resolved rapidly after heart valve replacementof the stenosed aortic valve. The coagulationabnormality, the study poses, is possibly caused by the increased breakdown of the very large von Willebrand factormolecule by its natural catabolicenzyme (named ADAMTS13) under conditions of high shear stressaround the valve.

Therapy

Symptomatic treatment can be given in the form of blood transfusions. Desmopressin(DDAVP) releases Factor VIIIstorage pools and is used in mild to moderate cases of von Willebrand disease. Other approaches, including estrogentherapy, are discussed in the angiodysplasia article.

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.

History

The syndrome was originally described in 1958by Dr Edward C. Heyde, an internistliving and working in Vancouver, WAin a letter to the New England Journal of Medicine. He reported ten patients with the association.

The statisticalassociation was proven before there was any clue why patients with aortic valve stenosis had a higher occurrence of colonic bleeding. Already in 1958, a letter in the New England Journal of Medicine (Goldman et al) reported an odds ratioof almost 3.0 between the two diseases.

References

Goldman MJ. Aortic stenosis and gastrointestinal bleeding. N Engl J Med 1958;259:941.
Heyde EC: Gastrointestinal bleeding in aortic stenosis. N Engl J Med 1958;259:196.
Vincentelli A, Susen S, Le Tourneau T, et al. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med2003;349:343-9. PMID 12878741
Warkentin TE, Moore JC, Morgan DG. Aortic stenosis and bleeding gastrointestinal angiodysplasia: Is acquired von Willebrand's disease the link? Lancet1992;340:35-7. PMID 1351610.
Warkentin TE, Moore JC, Morgan DG. Resolution of bleeding from gastrointestinal angiodysplasia and von Willebrand syndrome type 2A after aortic valve replacement. N Engl J Med2002;347:858-9. PMID 12226167.
Warkentin TE, Moore JC, Anand SS, Lonn EM, Morgan DG. Gastrointestinal bleeding, angiodysplasia, cardiovascular disease, and acquired von Willebrand syndrome. Transfus Med Rev 2003;17:272-86. PMID 14571395.