PICK YOUR POISON – TO CLOT OR TO BLEED?

Case Presentation: Heyde syndrome is a rare multisystem disorder characterized by the triad of aortic stenosis (AS), gastrointestinal bleeding, and acquired von Willebrand syndrome (AVWS). The pathophysiology of AVWS in Heyde syndrome is thought to be due to the increased circulatory shear forces resulting from AS, leading to cleavage of large VWF multimers and loss of platelet mediated homeostasis [1,2]. In addition to AS, dysfunction of prosthetic valves can be identified as the cause for AVWS. A significant reduction in high-molecular-weight multimers of von Willebrand factor (VWF) occurs in 20% to 70% of AS patients and gastrointestinal bleeding is observed in approximately 1% to 3% of cases in this age group [1]. It is a treatable condition with management of the GI bleeding and aortic valve replacement. However, we present a case of Heyde syndrome in circumstance of a previously replaced mechanical aortic valve.

Discussion: An 82-year-old female with a mechanical aortic valve presented with dark stools for past 3 days. Her aortic valve was replaced in 1977, and on coumadin since with INR range of 2.5-3.5 due to comorbidities and high-risk features. Initial investigations in the ED revealed positive FOBT with severe anemia (Hgb 5.3 g/dL) and an elevated supratherapeutic INR (3.8). LDH was 263 and haptoglobin was 97, ruling out hemolytic anemia. Reticulocyte count was 5%, favoring adequate compensation for blood loss and no signs of bone marrow dysfunction. The patient received two units of platelet-rich blood cells while warfarin was temporarily held. The gastroenterology team was consulted for endoscopy and colonoscopy as there was a high suspicion for an acute GI bleed. During colonoscopy, angiodysplasias were identified and successfully ablated, raising suspicion of Heyde syndrome. Due to the length of time of mechanical valve placement, and new onset angiodysplasia, echocardiogram was done to assess functionality of valve. It revealed an increased aortic velocity across the prosthesis with mean gradient of 24.5 mmHg and a max gradient of 40.6mmHg. The aortic valve area was 1cm/s2, suggestive of a poorly functioning valve. Patient was hemodynamically stabilized and discharged, with no bleeding after monitoring for several days.

Conclusions: The management of Heyde’s syndrome often involves aortic valve replacement, which ultimately resolves the patient’s AS and AVWS. In this case, the dilemma was that the patient already had a mechanical valve and being on anticoagulation complicated the approach further. In this case, a Gastrointestinal Bleeding Scan (GIBS) could have been an appropriate alternative to colonoscopy and becoming subtherapeutic. The downside to this alternative is that the patient would still need a therapeutic INR to undergo the ablation if bleeding is found on scan. The question of converting the mechanical valve to a bioprosthetic valve to eliminate long-term anticoagulation arises. While converting the valve into a bioprosthetic valve will mitigate the anticoagulation challenge in our patient, it presents significant risks for our patient. The choice between the two brings into question the patient’s end-of-life goals, as a bioprosthetic valve typically lasts 15 years, and we must discuss with our patient would want to endure a biosynthetic replacement in her late 90’s. Alternatively, repair of the mechanical valve or replacement may also present its risks similar those stated above. Discussions among several disciplines are vital in guiding treatment decisions.