Premium
Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case‐series
Author(s) -
Icheva V.,
NowakMachen M.,
Budde U.,
Jaschonek K.,
Neunhoeffer F.,
Kumpf M.,
Hofbeck M.,
Schlensak C.,
Wiegand G.
Publication year - 2018
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/jth.14208
Subject(s) - medicine , von willebrand factor , cardiopulmonary bypass , von willebrand disease , cardiac surgery , perioperative , heart disease , cardiology , incidence (geometry) , surgery , anesthesia , platelet , physics , optics
Essentials Bleeding complications during congenital heart disease surgery in neonatal age are very common. We report the perioperative incidence of acquired von Willebrand syndrome (aVWS) in 12 infants. aVWS was detected in 8 out of 12 neonates and infants intraoperatively after cardiopulmonary bypass. Ten patients received von Willebrand factor concentrate intraoperatively and tolerated it well.Summary Background Cardiac surgery of the newborn and infant with complex congenital heart disease ( CHD ) is associated with a high rate of intraoperative bleeding complications. CHD ‐related anatomic features such as valve stenoses or patent arterial ducts can lead to enhanced shear stress in the blood stream and thus cause acquired von Willebrand syndrome (aVWS). Objective To evaluate the intraoperative incidence and impact of aVWS after cardiopulmonary bypass (CPB) in neonates and infants with complex CHD. Patients/Methods We conducted a survey of patients aged < 12 months undergoing complex cardiac surgery in our tertiary referral center. Twelve patients, whose blood samples were analyzed for aVWS before CPB and immediately after discontinuation of CPB on a routine basis, were eligible for the analysis. von Willebrand factor antigen ( VWF :Ag), ristocetin cofactor activity ( VWF : RC o), collagen binding activity ( VWF : CB ), VWF :multimers and factor VIII activity ( FVIII :C) were determined. Results aVWS was diagnosed by VWF multimer analysis in 10 out of 12 patients (83%) prior to surgery and intraoperatively at the end of CPB in 8 out of 12 patients (66%). Ten patients received VWF / FVIII concentrate intraoperatively as individual treatment attempts during uncontrolled bleeding. They tolerated it well without intraoperative thrombotic events. One patient suffered a transient postoperative cerebral sinuous vein thrombosis. Conclusions aVWS is of underestimated incidence in complex CHD surgery. These data may offer a new approach to reduce the risk of severe bleedings and to achieve hemostasis during high‐risk pediatric cardiac surgery by tailoring the substitution with von Willebrand factor concentrate.