Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support

Kubicki, Rouven; Stiller, Brigitte; Kroll, Johannes; Siepe, Matthias; Beyersdorf, Friedhelm; Benk, Christoph; Hohn, R B; Grohmann, Jochen; Fleck, Thilo; Zieger, Barbara

doi:10.1093/ejcts/ezy408

Cited by 17 publications

(22 citation statements)

References 23 publications

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“…Acquired VWF syndrome occurs during ECMO due to a loss of high molecular weight VWF multimers from shear stress. Disrupting VWF multimers is associated with increased bleeding complications (45, 46).…”

Section: Hemostatic Alterations During Ecmo—ecmo Induced Coagulopathymentioning

confidence: 99%

Anticoagulation in Neonatal ECMO: An Enigma Despite a Lot of Effort!

2019

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Extracorporeal membrane oxygenation (ECMO) is a valuable modality used to support neonates, children, and adults with cardiorespiratory failure refractory to conventional therapy. It requires use of anticoagulation to prevent clotting in the extracorporeal circuit. Balancing bleeding from excessive anticoagulation with thrombotic risk remains a difficult aspect of ECMO care. Despite many advances in ECMO technology, better understanding of the coagulation cascade and new monitoring schemes to adjust anticoagulation, bleeding and thrombosis remain the most frequent complications in ECMO and are associated with morbidity and mortality. In neonates, ECMO is also complicated by the immature hemostatic system, laboratory testing norms which are not specific for neonates, lack of uniformity in management, and paucity of high-quality evidence to determine best practices. Traditional anticoagulation focuses on the use of unfractionated heparin. Direct thrombin inhibitors are also used but have not been well-studied in the neonatal ECMO population. Anticoagulation monitoring is complex and currently available assays do not take into account thrombin generation or platelet contribution to clot formation. Global assays may add valuable information to guide therapy. This review provides an overview of hemostatic alterations, anticoagulation, monitoring and management, novel anticoagulant use, and circuit modifications for neonatal ECMO. Future considerations are also presented.

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Section: Hemostatic Alterations During Ecmo—ecmo Induced Coagulopathymentioning

confidence: 99%

Anticoagulation in Neonatal ECMO: An Enigma Despite a Lot of Effort!

2019

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“…All patients under ECMO support develop severe AVWS within 1 hour and recover rapidly after weaning from the device 9. In a pediatric cohort including 11 newborns on ECMO/extracorporeal life support, we observed AVWS with 100% prevalence 10. We acknowledge that Meyer et al did not find changes in the VWF antigen with their experimental setup, but this functional defect of VWF can only be detected by specialized methods such as VWF collagen binding capacity assay or VWF mul-…”

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confidence: 63%

Contact activation and acquired von Willebrand syndrome during neonatal extracorporeal circulation

Schlagenhauf

Zieger

Muntean

2020

Journal of Thrombosis and Haemostasis

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itive aPL) 7 and that aPS/PT more than aβ2GPI is responsible for LA activity in these patients. 8 Finally, if aCL and aβ2GPI antibodies are of immunoglobulin G (IgG) isotype I would measure IgG aβ2GPI/domain 1 antibodies that are associated to thromboembolic events. 9 A tentative decision-making scheme to choose the appropriate anticoagulant treatment in young patients with venous thromboembolism is presented in Figure 1.

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“…A single-center observational study of 30 pediatric patients on mechanical circulatory support found a 100% prevalence rate of AvWS with no difference in bleeding events between extracorporeal membrane oxygenation (ECMO) and VAD-supported patients. 15 Although the majority of bleeding events in both groups were mediastinal, only the VAD group experienced gastrointestinal bleeding and intracranial hemorrhage. Seventyfive percent of VAD-supported patients experienced bleeding events with no difference between continuous flow and pulsatile devices.…”

Section: Acquired Von Willebrand Syndrome and Vad-supported Patientsmentioning

confidence: 91%

“…Seventyfive percent of VAD-supported patients experienced bleeding events with no difference between continuous flow and pulsatile devices. 15 Another retrospective review of severe bleeding events in the pediatric critical care unit found 14 cases associated with AvWS, 4 of whom were VAD-supported patients. The majority of bleeds were mediastinal or lower gastrointestinal.…”

Section: Acquired Von Willebrand Syndrome and Vad-supported Patientsmentioning

confidence: 99%

“…16 Many patients with AvWS remain asymptomatic until triggering situations such as surgery, trauma, or infection, an important consideration when taking a VAD-supported patient for a procedure. 15 Infection is a common trigger for bleeding in VAD-supported patients. Sometimes a clinically silent defect in vWF-related platelet dysfunction only manifests when additional hemostatic defects occur, such as commencing anticoagulation.…”

Section: Acquired Von Willebrand Syndrome and Vad-supported Patientsmentioning

confidence: 99%

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Management of a Pediatric Patient With a Left Ventricular Assist Device and Symptomatic Acquired von Willebrand Syndrome Presenting for Orthotopic Heart Transplant

Flannery

Kamra

Maeda

et al. 2020

Semin Cardiothorac Vasc Anesth

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We present the successful perioperative management of an 11-year-old patient presenting for heart transplant with a left ventricular assist device, symptomatic acquired von Willebrand syndrome, and recent preoperative intracranial hemorrhage. A brief review of the pathophysiology of acquired von Willebrand syndrome is included. As the number of pediatric patients supported with ventricular assist devices continues to increase, the management of symptomatic acquired von Willebrand syndrome during the perioperative period is an important consideration for anesthesiologists.

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Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support

Cited by 17 publications

References 23 publications

Anticoagulation in Neonatal ECMO: An Enigma Despite a Lot of Effort!

Anticoagulation in Neonatal ECMO: An Enigma Despite a Lot of Effort!

Contact activation and acquired von Willebrand syndrome during neonatal extracorporeal circulation

Management of a Pediatric Patient With a Left Ventricular Assist Device and Symptomatic Acquired von Willebrand Syndrome Presenting for Orthotopic Heart Transplant

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