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Elevated preoperative von Willebrand Factor is associated with perioperative thrombosis in infants and neonates with congenital heart disease.

Elevated preoperative von Willebrand Factor is associated with perioperative thrombosis in infants and neonates with congenital heart disease.
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Hunt R, Hoffman CM, Emani S, Trenor CC, Emani SM, Faraoni D, Kimchi-Sarfaty C, Ibla JC,


Hunt R, Hoffman CM, Emani S, Trenor CC, Emani SM, Faraoni D, Kimchi-Sarfaty C, Ibla JC, (click to view)

Hunt R, Hoffman CM, Emani S, Trenor CC, Emani SM, Faraoni D, Kimchi-Sarfaty C, Ibla JC,

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Journal of thrombosis and haemostasis : JTH 2017 10 05() doi 10.1111/jth.13860
Abstract
BACKGROUND
The surgical repair of congenital heart lesions is frequently complicated by perioperative thrombosis of unclear etiology. An imbalance between von Willebrand Factor (VWF) and ADAMTS-13 is an emerging variable in thrombosis.

OBJECTIVES
To describe perioperative changes to VWF, ADAMTS-13 and NETosis and evaluate clinical and biochemical associations with postoperative thrombosis.

METHODS
Neonates and infants undergoing palliation or definitive repair of congenital heart disease (CHD) were recruited (n =133). Pre- and postoperative plasma levels of VWF, ADAMTS-13 and markers of NETosis were determined. Patients were followed for up to 30 days for the occurrence of thrombosis. Univariate and multivariate logistic regression analyses were conducted to identify variables associated with thrombosis.

RESULTS
We identified significant postoperative increases in VWF activity, VWF concentration, DNA-histone complexes and cell-free DNA with an overall decrease in ADAMTS-13 activity. Patients experiencing postoperative thrombotic events (9%) were characterized by surgery performed at lower intraoperative temperature, higher preoperative lactic acid levels and higher preoperative VWF activity and concentration. A multivariate logistic regression model identified preoperative VWF activity (odds ratio: 8.39 per IU/mL, 95% CI: 1.73-40.55) and transfusion of cryoprecipitate (odds ratio: 1.10 per mL/kg, 95% CI: 1.03-1.17) to be associated with thrombosis.

CONCLUSIONS
Pediatric patients undergoing surgical repair of congenital heart disease are exposed to high levels of VWF with diminished or minimal change to ADAMTS-13 in the immediate postoperative period. Elevated preoperative VWF activity is associated with postoperative thrombosis in pediatric CHD. This article is protected by copyright. All rights reserved.

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