Abstract Number: PB0475
Meeting: ISTH 2022 Congress
Background: Venoarterial Extracorporeal Membrane Oxygenation (VA-ECMO) ensures a stable hemodynamic state in patients with life-threatening cardiac failure. Although it requires anticoagulation therapy for prevention of thrombotic events, several ECMO intrinsic mechanisms result in the development of Acquired von Willebrand disease (AvWD) and platelet dysfunction, with an increased bleeding tendency.
Aims: To report the case of a patient under VA-ECMO with AvWD associated bleeding.
Methods: A 54-year-old female, with several cardiovascular comorbidities, received VA-ECMO support after an ST-elevation myocardial infarction, Killip Class IV. While on a heart transplant waiting list, unfractionated heparin (UFH) was administered with minimal bleeding. During transplant surgery she required massive transfusion and, 5 days later, UFH was interrupted and a re-sternotomy was performed due to a large hemothorax. After stabilization, UFH was briefly reintroduced, but consequent bleeding through thoracic drains required transfusion and hemostatic treatment. von Willebrand Factor (vWF) antigen (Ag) and vWF ristocetin cofactor (RCo) were determined (respectively, 2.81 and 1.61UI/mL; vWF:RCo/Ag ratio 0.57), while an angio-computed tomography indicated that, besides several thoracic hematomas, an acute pulmonary embolism (PE) occurred.
Nonetheless, clinical evolution was favorable and, on day 57, ECMO was removed. vWF:RCo and vWF:Ag were determined once more (2.06 and 1.96UI/mL; ratio 0.95).
Results: Forty four days after ECMO implementation, the patient presented with serious bleeding and AvWD was investigated – despite the increased vWF:RCo and vWF:Ag, the low vWF:RCo/Ag ratio ( < 0.7), enforced the diagnosis of AvWD. Desmopressin was used as a therapeutic strategy, but the simultaneous diagnosis of PE excluded treatment with vWF concentrate due to its thrombotic risk. As expected, AvWD was resolved immediately after ECMO was discontinued.
Conclusion(s): The presence of a delicate balance between thrombotic and bleeding risks is challenging in patients undergoing. There are no current consensuses on bleeding management due to AvWD.
To cite this abstract in AMA style:Cibele D, Monteiro C, Silva S, Lopes M, Gonçalves L, Teixeira S, Machado I, Carvalho M, Roncon-Albuquerque R, Koch C. Acquired von Willebrand Disease in a Patient Undergoing Extracorporeal Membrane Oxygenation [abstract]. https://abstracts.isth.org/abstract/acquired-von-willebrand-disease-in-a-patient-undergoing-extracorporeal-membrane-oxygenation/. Accessed September 26, 2022.
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