Acquired von Willebrand's Syndrome in Congenital Heart Disease

Icheva, Vanya; Ebert, J.; Budde, U.; Wiegand, G.; Schober, Sarah; Engel, Juliane; Kumpf, Michael; Jaschonek, K.; Neunhoeffer, F.; Michel, Jörg; Schlensak, C.; Hofbeck, M.; Magunia, Harry

doi:10.1055/s-0042-1743019

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“…Hunt et al described lower postoperative levels of ADAMTS13 in the perioperative period of neonates and infants with CHD (33), which should be taken into account when considering VWF concentrate supplementation. In this context, the introduction of additional routine laboratory parameters such as the VWF:GPIbM/VWF:Ag ratio is a promising possibility for a timely aVWS diagnosis in the perioperative course of paediatric CHD patients (34). In our opinion, implementation of methods for rapid aVWS detection is necessary and should be available in centers performing neonatal cardiac surgery and providing extracorporeal support.…”

Section: Discussionmentioning

confidence: 99%

Acquired von Willebrand syndrome is common in infants with systemic-to-pulmonary shunts: Retrospective case-series

Icheva

Budde²,

Magunia

et al. 2022

Front. Pediatr.

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BackgroundAlthough acquired von Willebrand syndrome (aVWS) has been described in congenital heart disease before, anatomical features leading to aVWS with characteristic reduction or loss of high molecular weight von Willebrand multimers (HMWM) are not well known. This study assesses the prevalence and effects of aVWS in infants with systemic-to-pulmonary shunts (SPS).MethodsThis retrospective single-center study analyzes diagnostic data of infants with complex congenital heart defects requiring palliation with SPS. During the study period between 12/15–01/17 fifteen consecutive patients were eligible for analysis. Results of von Willebrand factor antigen (VWF:Ag), collagen binding activity (VWF:CB) and von Willebrand factor multimer analysis were included.ResultsIn all 15 patients with SPS an aVWS could be found. Blood samples were collected between 5 and 257 days after shunt implantation (median 64 days). None of the patients demonstrated increased bleeding in everyday life. However, 6 out of 15 patients (40%) showed postoperative bleeding complications after SPS implantation. Following shunt excision multimeric pattern normalized in 8 of 10 (80%) patients studied.ConclusionsThis study shows that in patients undergoing SPS implantation aVWS might emerge. Pathogenesis can be explained by shear stress resulting from turbulent flow within the shunt. Knowledge of aVWS existence is important for the consideration of replacement therapy with von Willebrand factor containing products and antifibrinolytic treatment in bleeding situations. Implementation of methods for rapid aVWS detection is required to achieve differentiated hemostatic therapy and reduce the risk of complications caused by empiric replacement therapy.

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Section: Discussionmentioning

confidence: 99%