Winning Abstracts from the 2010 Medical Student Abstract Competition: The Sound Of Bleeding
Author: Hailey Vincent, MD
Resident, Scott & White Hospital
Texas A&M Health Science Center College of Medicine, Class of 2010
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 aortic stenosis.
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.