C63-10 Diffuse Alveolar Hemorrhage in the Setting of Cardiogenic Pulmonary Edema: Compounding Effect of Acquired von Willebrand Deficiency and Acute Pulmonary Venous Hypertension

Abstract Introduction Acquired von Willebrand factor(vWF) deficiency in aortic stenosis is caused by shear-induced proteolysis of vWF. This results in a selective loss of high-molecular-weight vWF multimers, producing a hemostatic defect causing bleeding. It commonly manifests as mucocutaneous bleeding and is particularly associated with gastrointestinal bleeding. We present a case where diffuse alveolar hemorrhage(DAH) was the bleeding site in the setting of cardiogenic pulmonary edema due to severe aortic stenosis(AS) and mitral regurgitation(MR) in a patient with acquired vWF deficiency. Case A 73-year-old male with a history of atrial fibrillation, hyperlipidemia, and recently diagnosed prostate cancer presented with multiple episodes of hemoptysis, progressive dyspnea, and acute hypoxic respiratory failure. Evaluation revealed pulmonary edema secondary to severe AS and MR. He was noted to have anemia requiring multiple blood transfusions. Laboratory workup showed decreased vWF antigen levels, reduced vWF activity, and low Factor VIII activity, confirming the diagnosis of acquired von Willebrand factor deficiency. Other potential causes of DAH, including connective tissue disorders, vasculitis, and antiphospholipid syndrome, were excluded. He was deemed not a candidate for aortic valve replacement due to his multiple comorbidities and he ultimately succumbed to cardiogenic shock. Discussion The bleeding tendency of vWF deficiency is compounded by high pulmonary venous pressure from left ventricular failure as a result of AS and MR which led to alveoli being the bleeding site. This case highlights two compounding mechanisms - change in hemostatic and hydrostatic pressures resulting in alveolar edema and diffuse alveolar hemorrhage. This case emphasizes the need to recognize DAH as a potential bleeding manifestation in patients with severe AS and acquired vWF deficiency, especially in the context of cardiogenic pulmonary edema. This abstract is funded by: NA