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Author: Milla Kviatkovsky, Nova Southeastern
University College of Osteopathic Medicine, Class of 2014.
Introduction: In 1958, Heyde published 10 cases
describing the association of aortic stenosis (AS) and iron
deficiency anemia due to arteriovenous malformations (AVMs) of the
gastrointestinal tract. The postulated link between AS and anemia
is secondary to an acquired type IIA von Willebrand's Disease (vwD)
via breakdown of high molecular weight mul timers of von Willebrand
Factor (HvwF) across the stenotic valve with subsequent anemia
secondary to the development of Gastrointestinal (GI) bleeding from
angiodysplasia. Although this association is still controversial,
research suggests an Odds Ratio of 4.5 for the association of
angiodysplasia with AS and case reports demonstrate cessation of GI
bleeding via Aortic Valve Replacement (AVR). Case Report: We
present a case of a 74 year-old male who presented with chest pain
and shortness of breath and found to be anemic (hemoglobin/
hemocrit (H/H): 9.8/28.6). Patient had a history of frequent
admissions for angina exacerbated by Hb <10 which consistently
improved via blood transfusion. His GI bleeds were treated via
cauterization multiple times with suspected diagnoses ranging from
hematopoietic disorder, myelodysplastic disorder to anemia
secondary to renal failure. Further review of the patient's chart
revealed that he received his first transfusion in June 2007 during
initial presentation with the aforementioned symptoms when a
transthoracic echocardiogram (TTE) from 2007 suggested only mild
aortic valve sclerosis. During this hospital stay, he received
blood transfusions with normalization of his H/H and temporary
resolution of symptoms. During his admis sion in 2013,
transesophageal echocardioagraphy (TEE) was performed which
demonstrated an aortic valve area (AVA) of 0.8 cm2. Six years and
56 transfusions later, the newly diagnosed severe AS finally raised
suspicion for the diagnosis of Heyde Syndrome. Additional
laboratory findings revealed a prolongued collagen-epinephrine and
collagen-ADP time suggestive for diagnosis of acquired vwD. This
patient was subsequently treated via AVR with resolution of
bleeding and now stable H/H. Discussion: Research shows that the
mean trans-valvular pressure gradient is correlated with the degree
of loss of HvwF multimers and studies indicate that aortic valve
replacement (AVR) has reversed the aforementioned laboratory
abnormalities by first postoperative day. Current indications for
AVR include those patients with severe AS (defined by AVA <1.0
cm2) who are symptomatic or those with severe stenosis who fulfill
various other criteria- none of which include patients suffering
from an associated bleeding disorder. As evide nced in our patient,
a bleeding disorder secondary to stenosis may present even with
mild valvular disease. Because valve replacement is indicated as
curative in this population, severity of stenosis and/ or gradient
should not be sole criteria for patient selection. We recommend
further review of symptoms and clinical parameters to establish
separate criteria for AVR in this population with efforts to raise
early clinical suspicion and improve outcomes.
February 2015 Issue of IMpact