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Lower incidence of procoagulant abnormalities during follow-up after creation of the Fontan circulation in children

Published online by Cambridge University Press:  01 April 2009

Olof Rask ,
Katarina Hanséus ,
Rolf Ljung ,
Karin Strandberg  and
Erik Berntorp
Olof Rask*
Affiliation:
Department of Paediatrics, Malmö University Hospital, Malmö, Sweden
Katarina Hanséus
Affiliation:
Department of Paediatrics, Lund University Hospital, Lund, Sweden
Rolf Ljung
Affiliation:
Department of Paediatrics, Malmö University Hospital, Malmö, Sweden
Karin Strandberg
Affiliation:
Department of Laboratory Medicine, Malmö University Hospital, Malmö, Sweden
Erik Berntorp
Affiliation:
Centre for Thrombosis and Haemostasis, Malmö University Hospital, Malmö, Sweden
*
Correspondence to: Olof Rask, Department of Paediatrics, University Hospital, 205 02 Malmö, Sweden. Tel: +46 40-33 16 64. Fax: +46 40-336226; E-mail: olof.rask@med.lu.se
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Abstract

Objective

Children who undergo surgery for complex congenital cardiac disease are reported to be at increased thrombotic risk. Our aim was to evaluate long-term changes in the haemostatic system after surgery, to compare markers of activated coagulation in children having surgery with those in a healthy control population, and to relate them to adverse clinical outcome.

Patients and methods

We studied, prior to surgery, the coagulation profiles of a cohort of 28 children admitted for a modified Fontan operation, studying them again after a period of mean follow-up of 9.6 years. Median age at the time of final surgery was 18.5 months, with a range from 12 to 76 months. We compared generation of thrombin, and levels of the activated protein C-protein C inhibitor complex to controls at follow-up. Thrombophilia and clinical outcome were evaluated.

Results

At long-term follow-up, a lower incidence of procoagulant abnormalities was observed compared to that before surgery. Of 27 patients, 3 (11%), but none of 45 controls, had levels of activated protein C-protein C inhibitor complex above the reference range. There were no significant differences in generation of thrombin between patients and controls. No thrombotic events were recorded, and the patients were generally in good clinical condition.

Conclusions

Overall, haemostasis appeared to be in balance, and less prothrombotic, after surgery. A subset of the cohort did show indications of activated coagulation. The current therapeutic approach seems to be sufficient to protect the majority of patient. New tests of global coagulation, nonetheless, may be helpful in improving identification of individuals at increased thrombotic risk.

Keywords

Activated Protein C Resistanceheart defectscongenitalprotein C Inhibitorrisk factorsthrombinthromboembolism
Type
Original Article
Information
Cardiology in the Young , Volume 19 , Issue 2 , April 2009 , pp. 152 - 158
Copyright
Copyright © Cambridge University Press 2009

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