CHSU Discovery

Abstract:
Heyde Syndrome is reflected by a triad of aortic stenosis, acquired von Willebrand Syndrome, and recurrent gastrointestinal bleeding from intestinal angiodysplasia. Our case reflects a scenario of a patient with severe aortic stenosis and severe lower gastrointestinal bleeding that was difficult to stabilize with blood transfusions, in the setting of inconclusive von Willebrand syndrome work up.

Patient is an 85-year-old male with history of severe aortic stenosis, hypertension, gastric ulcers, diverticulosis, atrial fibrillation, HFrEF with EF 35-40%, CAD s/p cardiac stents x3 who presented to the ED for bright red blood per rectum associated with dyspnea, chest pain, and dizziness. Two weeks prior, patient had polypectomy from ascending colon resected. At time of admission, blood pressure was 96/51, with hemoglobin of 8.3 dL. CTA of abdomen/pelvis revealed contrast extravasation consistent with active bleeding in cecum. Home aspirin and clopidogrel were stopped.Hemoglobin continued to drop despite multiple blood transfusions.

Colonoscopy showed blood-tinged effluent in the transverse colon, ascending colon, and cecum. No active bleeding was noted, including from endoclips from previous polypectomy site. Three cherry red spots were noted without active bleed, and were coagulated. Von Willebrand syndrome type 2A workup initiated for Factor VIII activity, vW Ag, vWF multimeric antigen were found to be inconclusive after ~10 day result turnaround. Patient’s condition eventually improved. Cardiology was consulted and advised patient to complete transcatheter aortic valve replacement (TAVR) outpatient. Hemoglobin levels was advised to keep > 9.0 dL. Clopidogrel was resumed due to greater risk of stent thrombosis than bleeding.

This patient met 2 of the 3 criteria for Heyde Syndrome: aortic stenosis and recurrent lower gastrointestinal GI bleeding with angiomatosis. Although workup for von Willebrand Syndrome Type 2A was inconclusive, the time for pending results may be reduced with alternate modalities. Furthermore, blood transfusions is associated with increased VWF and thus levels may be falsely elevated in our patient at the time of testing. Platelet function analyzer (PFA), for example are more sensitive and offer faster results when compared to confirming Von Willebrand Syndrome in Heyde Syndrome cases.
Researches suggest the repair of aortic stenosis is associated with higher rates of lower GI bleeding resolution in patients with Heyde Syndrome. This patient is to follow up outpatient for a TAVR with cardiology.

As evidenced in our case, utilizing approaches such as PFA may offer faster therapeutics to patients when compared other diagnostic modalities such as vW Ag, vWF multimeric antigen. As such, it is important to consider modalities such as PFA when treating patients who have aortic stenosis and anemia from GI bleeding that is unresolving with transfusions.