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Author: Hailey Vincent, MD
Resident, Scott & White Hospital
Texas A&M Health Science Center College of Medicine, Class of
Heyde's syndrome was first reported in 1958 as a correlation
between aortic stenosis and gastrointestinal (GI) bleeding.
Recently, it has been associated with the triad of aortic stenosis,
gastrointestinal angiodysplasia, and acquired von Willebrand
syndrome. Several case studies and small series have appeared in
the literature, yet, 50 years later, there are no clinical
guidelines for evaluation of anemia or GI bleeding in patients with
An 83-year-old Caucasian female with known aortic stenosis and mild
anemia presented to the emergency department with right-sided chest
pain and dyspnea. She reported three days of worsening dyspnea on
exertion during activities of daily living. She denied
hematochezia, but did note dark stools related to iron
supplementation for anemia. A transesophageal echocardiogram showed
aortic stenosis, with a valve area of 1.1 cm2. Laboratory results
revealed significant anemia with a hemoglobin of 6.7 g/dL (MCV
109.5 fL), a drop from 11.8 g/dL just 4 weeks prior. She had
appropriate levels of B12 and folate, and iron levels were
consistent with iron replacement. She was admitted and received 4
units of packed red blood cells. GI bleeding was investigated with
endoscopy and colonoscopy, both with negative results. Outpatient
camera endoscopy showed a small, non-bleeding arteriovenous
malformation of the small bowel. Due to the patient's aortic
stenosis and presumed blood loss, anemia with a GI source, Heyde's
syndrome, was suspected. Von Willebrand antigen and factor VIII
showed marked elevations, while high molecular weight von
Willebrand multimers were decreased. The patient underwent aortic
valve replacement. Two months later, von Willebrand markers were
found to be within normal limits and the anemia resolved.
Heyde's syndrome is a consequence of aortic stenosis that can be
explained by the biochemical construction and fluid hemodynamics of
blood. Sheer stress across a stenotic valve opening damages the
high molecular weight multimers of von Willebrand factor (vWF),
leading to an acquired qualitative defect (Type 2A von Willebrand
syndrome). Angiodysplasia of the small bowel is common in the
elderly. Normally vWF is the first line of defense against this
lesion by its action to initiate platelet adherence to damaged
endothelium. Although reported in the literature, Heyde's syndrome
is infrequently diagnosed in clinical practice. The correlation
between aortic stenosis and GI bleeding needs to be recognized and
considered in the differential diagnosis, as this is a reversible
syndrome. GI bleeding in the presence of aortic stenosis merits
further investigation to determine if Heyde's syndrome is present.
Current ACC/AHA guidelines for aortic valve replacement doesn't
include Heyde's syndrome, but with the reversibility of the
bleeding it warrants possible inclusion.
November 2010 Issue of IMpact