Case Presentation: Our patient is a 66-year-old female who was admitted for shortness of breath and generalized weakness. She was severely anemic with hemoglobin of 6.5. Her EGD did not show any obvious evidence of bleeding. Colonoscopy in the past showed a large inflammatory mass consistent with ischemic colitis. Follow-up colonoscopy showed completely healed mucosa. She received 4 units of packed RBC transfusion. Small bowel capsule endoscopy showed few non bleeding angiectasia in the jejunum. No evidence of active over GI bleeding. She underwent echocardiogram, as the examination showed severe systolic murmur. The echocardiogram showed severe aortic stenosis with a valve area of 0.8 square centimeters. The patient was diagnosed with severe iron deficiency anemia and was discharged home under stable condition with iron supplementation.Patient had multiple hospitalization with recurrent GI bleed. She underwent repeat endoscopy and colonoscopy, which showed AVM. She underwent prosthetic aortic valve replacement in a tertiary center. Her hemoglobin has remained stable since then with no further episodes of bleeding.
Discussion: The association between aortic valve stenosis and intestinal angiodysplasia was first described in 1958 by Heyde and was named after him as Heyde’s syndrome. In 1971, they theorized that chronic hypoxia may cause sympathetic vasodilation and smooth muscle relaxation causing ectasia of the vessel wall. It is probably a result of an accelerated interaction of the platelets due to the turbulent flow through the stenosed aortic valve. This explains that they should be increased in the breakdown of high molecular weight multimers of Von Willebrand factor. This explains that bleeding disorder which is acquired type II A Von Willebrand syndrome.The loss of large VWF multimers affects both platelet adhesion and ADP-inducible platelet aggregation. Measuring the levels of high molecular weight multimers of Von Willebrand factor and dynamic platelet function testing may show coagulopathy. The goal standard exam is gel electrophoresis of Von Willebrand factor, showing the absence of large polymers.There have been reports which showed recurrence of GI bleeding even after aortic valve replacement and complete cessation of bleeding after replacement. Another issue was recurrent bleeding from excessive anticoagulation after valve replacement. In such a situation prosthetic valves became the better choice of replacement in patient with GI bleeding rather than the mechanical valve.
Conclusions: Although Iron deficiency anemia and aortic stenosis are common in elderly, the association is not usually recognized. Heyde’s syndrome is a complex disorder which results from interaction between the aortic stenosis, type IIA VonWillebrand factor and intestinal angiodysplasia. Aortic valve replacement rather than bowel surgery leads to long-term cessation of GI bleed from angiodysplasia.
