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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 Signs and symptoms
Aortic valve stenosis is a cause of heart failure and several other cardiac problems. In most patients, however, it goes unsuspected. A number of patients develops a bleeding tendency resembling that of platelet-derived coagulation problems, 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 transfusions and surgery to control bleeding.
2 Diagnosis
Angiodysplasias are usually identified on colonoscopyColonoscopy is the endoscopic examination of the large colon and the distal part of the small bowel with a fiber optic camera on a flexible tube passed through the anus. It may provide a visual diagnosis (e. ulceration, polyps) and grants the opportunity. In some patients, the cause of bleeding cannot be identified on colonoscopy, and only angiography of the colonic vasculature can identify the location of the angiodysplasias.
Aortic valve stenosis is identified on echocardiographyThe echocardiogram is an ultrasound of the heart. Using standard ultrasound techniques, two-dimensional slices of the heart can be imaged. The standard echocardiogram is also known as a transthoracic echocardiogram, or TTE. In this case, the echocardiogra ( ultrasoundMedical ultrasonography is an ultrasound-based imaging diagnostic technique used to visualize internal organs, their size, structure and their pathological lesions. Ultrasonography is widely utilized in medicine, primarily in gastroenterology, cardiology, studies of the heart), where a decreased Orifice Area of the aortic valve implies stenosis.
Routine testing for Von Willebrand disease (see below) generally fails to identify the coagulation abnormality, as only the high-molecular weight-multimers (HMWM) of von Willebrand factor are decreased. Only dynamic platelet function analysis (PFA) might be of some use in this respect.
3 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 2003 a study by Vincentelli et al finally gave an explanation for the remarkable phenomenon. It showed how the subtle form of von Willebrand disease present in Heyde syndrome patients resolved rapidly after heart 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.
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