Loba Alam, MD1, Abhishek Bhurwal, MD2
1Integrative Medicine, Atlantic Health System, Summit, NJ; 2Robert Wood Johnson Medical School, Rutgers University, New Brunswick, NJ
Introduction: The association between aortic stenosis (AS), acquired von Willebrand disease (aVWD) type IIA, and intestinal arteriovenous malformations (AVMs) is termed Heyde’s Syndrome. We report two patients with severe AS and recurrent GI bleeding from AVMs. The aim of this case series is to recognize this syndrome and take a multidisciplinary approach in the management of these patients.
Case Description/Methods: Case 1: 67-year-old white male with a history of hypertension and severe aortic stenosis who presented to the hospital for melanotic stools, dyspnea on exertion, and worsening fatigue. Labs revealed acute blood loss anemia with hemoglobin of 6.3g/dL. Balloon enteroscopy demonstrated small-bowel AVMs, duodenitis, and bleeding from proximal jejunum (Figure 1). The lesions were treated with argon laser and patient was ultimately discharged. Patient was readmitted within a week for symptomatic anemia. Endoscopy revealed two small erosions in the duodenum which were clipped. As repeat echocardiogram showed severe aortic stenosis with valve area of 0.63cm2 and peak gradient of 76 mmHg, patient was accepted for TAVR given the significant association of AVMs and AS. Case 2: 70-year-old male with history of coronary artery disease s/p stent, severe aortic stenosis, and multiple GI bleeds who presented with melanotic stools and hemoglobin of 6.5g/dL. Capsule endoscopy showed multiple AVMs in the small intestine. Balloon enteroscopy confirmed 5 AVMs in the stomach, 1 duodenal AVM, and 2 AVMs in the jejunum- all of which were treated with argon laser (Figure 1). Patient was subsequently discharged home.
Discussion: In 1958, E. C. Heyde reported an association between AVMs of the GI tract with AS. The bleeding risk seems to correlate with the severity of AS causing increased wall shear forces. Such an environment predisposes to bleeding in patients due to a) decreased blood flow causing GI mucosal ischemia, and b) acquired VWF dysfunction due to breakdown of VWF multimers in high shear force environment causing coagulopathy. This case series impacts clinician decision making by highlighting 1) Heyde’s syndrome should be considered in the differential in patients with concurrent GI bleeding and severe AS, 2) electrophoresis of vWF multimers should be obtained, and 3) TAVR should be considered in hopes of cessation of coagulopathy and AVMs.
Citation: Loba Alam, MD; Abhishek Bhurwal, MD. P1354 - THE LINK BETWEEN AORTIC STENOSIS AND RECURRENT GASTROINTESTINAL BLEEDING: HEYDE’S SYNDROME. Program No. P1354. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.