PB0475 - Acquired von Willebrand Disease in a Patient Undergoing Extracorporeal Membrane Oxygenation

Monday, July 11, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: In-Person

Presenting Author(s)

Diana Cibele, MD

Immunohemotherapy Department, Centro Hospitalar Universitário de São João
Centro Hospitalar Universitário São João
Porto, Porto, Portugal

Co-Author(s)

CM

Carla Monteiro

Attending
Center of Thrombosis and Haemostasis, Congenital Coagulopathies Reference Centre, Department of Immunohemotherapy, Centro Hospitalar Universitário São João
Porto, Porto, Portugal
SS

Sofia Silva

Attending
Department of Emergency and Intensive Care Medicine, Centro Hospitalar Universitário São João
Porto, Porto, Portugal
ML

Manuela Lopes

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Porto, Porto, Portugal
LG

Luciana Gonçalves

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João
Porto, Porto, Portugal
Sofia Teixeira, MA

MD, Immunohemotherapy Department
Centro Hospitalar Universitário de São João
Porto, Porto, Portugal
Ines Machado, MSc

MD, Immunohemotherapy Department
Centro Hospitalar Universitário de São João
Porto, Porto, Portugal
MC

Manuela Carvalho

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João
Porto, Porto, Portugal
RR

Roberto Roncon-Albuquerque

Attending
Department of Emergency and Intensive Care Medicine, Centro Hospitalar Universitário São João,
Porto, Porto, Portugal
CK

Carmo Koch

MD
Immunohemotherapy Department, Centro Hospitalar Universitário de São João, Porto, Portugal
Porto, Porto, Portugal

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.