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Published on: October 24, 2018
Acquired von Willebrand disease in children undergoing extracorporeal membrane oxygenation: a prospective
Joppe G Drop1, Enno D Wildschut2, Moniek P M de Maat3
1Department of Paediatrics, Division of Paediatric Hematology, Erasmus Medical Center Rotterdam - Sophia Children's Hospital, Rotterdam, the Netherlands; Department of Paediatrics, Division of Pediatric Intensive care and Pediatric surgery, Erasmus Medical Center Rotterdam - Sophia Children's Hospital, Rotterdam, the Netherlands.
Insights
Children on extracorporeal membrane oxygenation (ECMO) frequently develop acquired von Willebrand disease (AVWD). This condition develops quickly after ECMO initiation and resolves upon cessation, but it does not appear to cause major bleeding complications.
Area of Science:
- Pediatric critical care medicine
- Hematology
- Cardiopulmonary support
Background:
- Extracorporeal membrane oxygenation (ECMO) is vital for pediatric patients with severe cardiac or pulmonary failure.
- Bleeding complications are frequent during ECMO support.
- Acquired von Willebrand disease (AVWD) is a potential contributor to these bleeding issues.
Purpose of the Study:
- To determine the incidence and temporal changes of AVWD in children during the first 14 days of ECMO.
- To assess the relationship between AVWD and bleeding complications in this population.
Main Methods:
- Prospective observational study of pediatric patients (0-17 years) on ECMO.
- Serial blood sampling to analyze von Willebrand factor (VWF) parameters and multimer patterns.
- AVWD defined by loss of high-molecular weight multimers or specific VWF ratio thresholds.
Main Results:
- All 50 patients developed AVWD during ECMO.
- VWF parameters and high-molecular weight multimers decreased early in ECMO and normalized within 24 hours of cessation.
- AVWD incidence and profile were similar in patients with and without major bleeding.
Conclusions:
- Pediatric patients on ECMO commonly develop AVWD.
- AVWD onset is rapid post-ECMO initiation and resolution is quick after ECMO cessation.
- AVWD appears to be an independent finding and not directly linked to major bleeding events.
Background:
Extracorporeal membrane oxygenation (ECMO) provides cardiopulmonary support for children with severe cardiac and/or pulmonary failure. The incidence of bleeding complications during ECMO support is high. Acquired von Willebrand disease (AVWD) might contribute to the development of bleeding complications.
Objective:
To study the incidence and longitudinal profile of AVWD during the first 14 days of ECMO support in children and to investigate the association between AVWD and bleeding complications.
Methods:
This prospective observational study included pediatric patients (0-17 years) receiving ECMO. Blood was sampled prior to and after ECMO start, daily and 12 to 24 hours after stopping ECMO. von Willebrand factor (VWF) parameters and multimer patterns were determined. Clinical data were collected for each patient. AVWD was defined as loss of high-molecular weight multimers (ie, decreased compared with baseline) or a VWF:collagen binding/VWF: antigen (Ag) ratio or VWF:activity/VWF:Ag ratio below 0.7.
Results:
All of 50 (100%) patients developed AVWD during ECMO. The VWF:collagen binding /VWF:Ag ratio, VWF:activity/VWF:Ag ratio, and high-molecular weight multimers decreased during the initial days and recovered to baseline level within 24 hours after stopping ECMO. The incidence and longitudinal profile of AVWD were similar in patients with and without major bleeding complications.
Conclusion:
Children receiving ECMO support commonly develop AVWD. AVWD develops rapidly after ECMO initiation and recovers quickly after ECMO cessation. Importantly, AVWD appears to be independent of major bleeding.
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