An Uncommon Presentation of Heyde Syndrome: Absence of Visible Endoscopic Angiodysplasia With Resolution After

Ronnie A Napoles1, Cristina Benites2, Gabriella Morey2

  • 1Department of Cardiology, HCA Florida Aventura Hospital, Aventura, FL, USA.

BACKGROUND Heyde syndrome (HS) is defined by the triad of aortic stenosis (AS), gastrointestinal bleeding, and acquired von Willebrand syndrome (avWS). Diagnosis can be challenging in elderly patients with multiple comorbidities, particularly when angiodysplasia is absent on endoscopy. We present a case illustrating the diagnostic process and therapeutic outcome, emphasizing that the absence of angiodysplasia should not exclude HS. CASE REPORT A 73-year-old woman with a history of AS, coronary artery disease, hypertension, type 2 diabetes, and anemia presented with 2 weeks of melena and 1 week of fatigue. She was pale on exam with a harsh systolic murmur radiating to the carotids. Laboratory evaluation showed hemoglobin 7.7 g/dL, microcytosis, and elevated troponin consistent with type II myocardial infarction due to demand ischemia. Prior endoscopic evaluations, including esophagogastroduodenoscopy, colonoscopy, and capsule studies, revealed melena but no bleeding source. Coagulation studies assessing von Willebrand factor activity were not available from the referring facility in this case. Transthoracic echocardiogram and cardiac catheterization confirmed severe AS (aortic valve area 0.4-0.7 cm2) with concomitant coronary artery disease. The patient underwent transcatheter aortic valve replacement (TAVR). At 6-month follow-up, she was asymptomatic, with resolution of anemia (hemoglobin 14 g/dL) and negative fecal occult blood testing. CONCLUSIONS Acquired von Willebrand factor deficiency diagnostic measures were unable to be provided from the facility at which she was originally admitted. This case underscores the importance of considering HS in patients with severe AS and recurrent gastrointestinal bleeding even when angiodysplasia is not visualized. Hemodynamic shear stress in AS leads to degradation of high-molecular-weight von Willebrand factor multimers, predisposing to bleeding. The resolution of anemia and bleeding after TAVR strongly supports the diagnosis. Early recognition and valve replacement are essential for effective management.

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