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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: [email protected]
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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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References

1.Monagle, P, Karl, TR. Thromboembolic problems after the Fontan operation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 36–47.CrossRefGoogle ScholarPubMed
2.Balling, G, Vogt, M, Kaemmerer, H, Eicken, A, Meisner, H, Hess, J. Intracardiac thrombus formation after the Fontan operation. J Thorac Cardiovasc Surg 2000; 119: 745–752.Google ScholarPubMed
3.Hanséus, K, Björkhem, G, Jögi, P, Sonesson, S-E. Thrombi and thromboembolism after bidirectional Glenn anastomosis, total cavopulmonary connection and the Fontan operation. Cardiol Young 1998; 8: 211–216.CrossRefGoogle Scholar
4.Kaulitz, R, Ziemer, G, Rauch, R, et al. Prophylaxis of thromboembolic complications after the Fontan operation (total cavopulmonary anastomosis). J Thorac Cardiovasc Surg 2005; 129: 569–575.CrossRefGoogle ScholarPubMed
5.Walker, HA, Gatzoulis, MA. Prophylactic anticoagulation following the Fontan operation. Heart 2005; 91: 854–856.CrossRefGoogle ScholarPubMed
6.Cromme-Dijkhuis, AH, Hess, J, Hahlen, K, et al. Specific sequelae after Fontan operation at mid- and long-term follow-up. Arrhythmia, liver dysfunction, and coagulation disorders. J Thorac Cardiovasc Surg 1993; 106: 1126–1132.CrossRefGoogle ScholarPubMed
7.Jahangiri, M, Shore, D, Kakkar, V, Lincoln, C, Shinebourne, E. Coagulation factor abnormalities after the Fontan procedure and its modifications. J Thorac Cardiovasc Surg 1997; 113: 989–992; discussion 992–983.CrossRefGoogle ScholarPubMed
8.Odegard, KC, McGowan, FX Jr, Zurakowski, D, et al. Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor VIII may predispose to thrombosis. J Thorac Cardiovasc Surg 2003; 125: 1260–1267.CrossRefGoogle ScholarPubMed
9.Cheung, EW, Chay, GW, Ma, ES, Cheung, YF. Systemic oxygen saturation and coagulation factor abnormalities before and after the fontan procedure. Am J Cardiol 2005; 96: 1571–1575.CrossRefGoogle ScholarPubMed
10.Strandberg, K, Svensson, A, Stenflo, J. Stabilyte tubes that contain strongly acidic citrate prevent in vitro complex formation between activated protein C and protein C inhibitor. Thromb Haemost 2003; 89: 947–949.CrossRefGoogle ScholarPubMed
11.Malm, J, Laurell, M, Dahlback, B. Changes in the plasma levels of vitamin K-dependent proteins C and S and of C4b-binding protein during pregnancy and oral contraception. Br J Haematol 1988; 68: 437–443.CrossRefGoogle Scholar
12.Strandberg, K, Kjellberg, M, Knebel, R, Lilja, H, Stenflo, J. A sensitive immunochemical assay for measuring the concentration of the activated protein C-protein C inhibitor complex in plasma: use of a catcher antibody specific for the complexed/cleaved form of the inhibitor. Thromb Haemost 2001; 86: 604–610.Google ScholarPubMed
13.Varadi, K, Negrier, C, Berntorp, E, et al. Monitoring the bioavailability of FEIBA with a thrombin generation assay. J Thromb Haemost 2003; 1: 2374–2380.CrossRefGoogle ScholarPubMed
14.Wright, EM, Royston, P. Calculating reference intervals for laboratory measurements. Stat Methods Med Res 1999; 8: 93–112.CrossRefGoogle ScholarPubMed
15.Andrew, M, Paes, B, Johnston, M. Development of the hemostatic system in the neonate and young infant. Am J Pediatr Hematol Oncol 1990; 12: 95–104.CrossRefGoogle ScholarPubMed
16.Andrew, M, Vegh, P, Johnston, M, Bowker, J, Ofosu, F, Mitchell, L. Maturation of the hemostatic system during childhood. Blood 1992; 80: 1998–2005.CrossRefGoogle ScholarPubMed
17.Strandberg, K, Stenflo, J, Nilsson, C, Svensson, PJ. APC-PCI complex concentration is higher in patients with previous venous thromboembolism with Factor V Leiden. J Thromb Haemost 2005; 3: 2578–2580.CrossRefGoogle ScholarPubMed
18.Watanabe, R, Wada, H, Sakakura, M, et al. Plasma levels of activated protein C-protein C inhibitor complex in patients with hypercoagulable states. Am J Hematol 2000; 65: 35–40.3.0.CO;2-1>CrossRefGoogle ScholarPubMed
19.Jahangiri, M, Kreutzer, J, Zurakowski, D, Bacha, E, Jonas, RA. Evaluation of hemostatic and coagulation factor abnormalities in patients undergoing the Fontan operation. J Thorac Cardiovasc Surg 2000; 120: 778–782.CrossRefGoogle ScholarPubMed
20.Rauch, R, Ries, M, Hofbeck, M, Buheitel, G, Singer, H, Klinge, J. Hemostatic changes following the modified Fontan operation (total cavopulmonary connection). Thromb Haemost 2000; 83: 678–682.Google ScholarPubMed
21.Barnes, C, Monagle, P. Haemostatic changes following the modified Fontan procedure. Thromb Haemost 2001; 86: 1341; author reply 1342.Google ScholarPubMed