Cardiology

Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support.



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Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support.

Eur J Cardiothorac Surg. 2018 Dec 22;:

Authors: Kubicki R, Stiller B, Kroll J, Siepe M, Beyersdorf F, Benk C, Höhn R, Grohmann J, Fleck T, Zieger B

Abstract
OBJECTIVES: Bleeding signs can become life-threatening complications in patients on mechanical circulatory support (MCS). Clinical phenotyping and comprehensive analyses of the cause of bleeding are, therefore, essential, especially when risk-stratifying patients during MCS workup. We conducted coagulation analyses and determined von Willebrand factor (VWF) parameters in a paediatric cohort on temporary extracorporeal life support, extracorporeal membrane oxygenation or long-term ventricular assist device support.
METHODS: We carried out an observational single-centre study including 30 children with MCS (extracorporeal life support, n = 13; extracorporeal membrane oxygenation, n = 5; and ventricular assist device, n = 12). We also assessed the acquired von Willebrand parameters of each study participant: collagen binding capacity (VWF:CB), the ratio of collagen-binding capacity to VWF antigen (VWF:CB/VWF:Ag) and high-molecular-weight VWF multimers. We also documented bleeding events, transfusion requirement, haemolysis parameters and surgical interventions.
RESULTS: All children developed AVWS (acquired von Willebrand syndrome) during MCS, usually during the early postoperative course. They presented no AVWS after device explantation. We detected a loss of high-molecular-weight VWF multimers, decreased VWF:CB/VWF:Ag ratios and reduced VWF:CB levels. Twenty of the 30 patients experienced bleeding complications; approximately 53% of them required surgical revision. There were no deaths due to bleeding during support.
CONCLUSIONS: The AVWS prevalence in paediatric patients on MCS is 100% regardless of the types of devices tested in this study. The bleeding propensity of AVWS patients widely varies.

PMID: 30590475 [PubMed – as supplied by publisher]

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