Winning Abstracts from the 2014 Medical Student Abstract Competition: The Bloody Aortic Stenosis: A Case of Heyde Snydrome

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.

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