Case Presentation: A 76 year-old male with a history of diverticulitis, T2DM, HTN, persistent atrial fibrillation and compensated alcoholic cirrhosis presented for recurrent gastrointestinal bleeding (GIB). He initially presented to an outside hospital six months prior for shortness of breath and found to be anemic requiring 4 units of packed red blood cells (PRBCs). Since that time, he has been hospitalized every one to two weeks with similar complaints, often anemic to a Hb of 5 to 6 requiring frequent blood transfusions. Prior multiple endoscopic and nuclear studies demonstrated blood throughout the colon and many gastric and small bowel arteriovenous malformations (AVMs) that were treated. He was transferred to our hospital for advanced intervention.
The patient endorsed intermittent episodes of dark bowel movements for five weeks, but denied frank bleeding. He also denied fever, chills, abdominal pain, chest pain, palpitations, diarrhea, syncopal episodes. On arrival to our hospital, he was hemodynamically stable with a blood pressure of 129/60mmHg and a Hb of 7.5 g/dL. Home medications of aspirin and ibuprofen were promptly stopped.
His exam was notable for an irregularly irregular heart rate and a loud V/VI systolic murmur heard best at the right upper sternal border. Capsule endoscopy showed multiple areas in the small bowel concerning for AVMs. Meanwhile, a TTE revealed an EF of 70%, severely enlarged left atrium, and a severely calcified aortic valve with a mean peak pressure of 106mmHg and a mean pressure gradient of 56mmHg. The aortic valve area was 0.5cm2, consistent with severe aortic stenosis (AS). Balloon valvuloplasty of the aortic valve was performed as a bridge towards a future transaortic valve replacement (TAVR). Subsequently, double balloon enteroscopy allowed for APC of 7 jejunal AVMs. Following his procedures, the patient did not show any evidence of continued gastrointestinal bleeding. His Hb was stable for 24 hours prior to discharge.
Discussion: Heyde’s syndrome refers to the triad of lower GIB from AVMs with aortic stenosis. The underlying pathophysiology is thought to be due to an increased trans-valvular shearing stress of Von Willebrand Factor (vWF) multimers, which are important in facilitating primary hemostasis. The then acquired vWF multimer deficiency predisposes to bleeding, especially in high flow areas such as AVMs. It is a widely under-recognized and life-threatening condition that should be considered in any patient, but especially in the elderly population, who presents with recurrent GIB.
Our patient had numerous admissions for recurrent lower GIB requiring multiple blood transfusions with preventable diagnostic and therapeutic interventions. Routine cauterization of AVMs with endoscopy only temporizes bleeding. However, valve replacement appears to offer long-term control as has been demonstrated by multiple studies. In this case, his significant systolic murmur prompted evaluation with TTE. The identification of severe AS from led to the decision to correct the valvulopathy via balloon valvuloplasty as a bridge to eventual TAVR.
Conclusions: Early recognition of Heyde’s Syndrome in elderly patients with recurrent lower GIB is crucially important. A thorough physical exam should be performed in all patients with GIB. With early consideration of Heyde’s Syndrome and correction of AS, a reduction in the rate of hospital readmissions, inpatient hospital days, blood product transfusions and invasive diagnostic and therapeutic interventions can be achieved.