Pediatric Cardiology

, Volume 27, Issue 1, pp 96–101 | Cite as

Ethnic Differences in Coagulation Factor Abnormalities After the Fontan Procedure



We tested the hypothesis that Chinese patients have a coagulation profile that is less prothrombotic than that of Caucasian counterparts after the Fontan procedure by determining the type and prevalence of anticoagulant and procoagulant deficiencies in Chinese patients and comparing the findings to those previously reported in Caucasian series. The liver function and coagulation factors were assessed in 21 ethnic Chinese patients, aged 17.0 ± 5.6 years, at 10.7 ± 4.0 years after the Fontan procedure. The results were compared to those of 21 age-matched Chinese controls with minor congenital heart disease. The prevalence of coagulation factor deficiencies in our patients was further compared to that reported in Caucasian patients. When compared with controls, patients had significantly lower protein C (p = 0.014), factors II (p = 0.024), V (p < 0.001), VII (p < 0.001), IX (p = 0.036), and X (p < 0.001), and higher bilirubin (p = 0.001) levels. The prevalence of protein C deficiency was 9.5%, whereas those of factor II, V, VII, IX, and X deficiencies were 0, 66.7, 9.5, 0, and 57.1%, respectively. When compared with Caucasian data, our data showed a significantly lower prevalence of protein C, total protein S, antithorombin III, factor II, and factor VII deficiencies. Furthermore, the previously reported increase in factor VIII levels was not found. In contrast, the prevalence of factor X deficiency was higher in our patients. This study provides the first evidence of ethnic differences in coagulation factor abnormalities after the Fontan procedure. The imbalance between procoagulant and anticoagulant pathways in Chinese patients favors a bleeding, rather than a thrombotic, tendency.


Fontan procedure Ethnic difference Coagulation 


  1. 1.
    Balling G, Vogt M, Kaemmerer H, et al. (2000) Intracardiac thrombus formation after the Fontan operation. J Thorac Cardiovasc Surg 119:745–751CrossRefPubMedGoogle Scholar
  2. 2.
    Bull K (1998) The Fontan procedure: lessons from the past. Heart 79:213–214CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Chan KY, Yuen ST, Ng TH, Ng WF (1991) An autopsy study of pulmonary thromboembolism in Hong Kong Chinese. Pathology 23:181–184CrossRefGoogle Scholar
  4. 4.
    Chan KY, Yuen ST, Wong MP (1997) Clinicopathological pattern of pulmonary thromboembolism in Chinese autopsy patients: comparison with Caucasian series. Pathology 29:263–266CrossRefGoogle Scholar
  5. 5.
    Chan LC, Bourke C, Lam CK, et al (1996) Lack of activated protein C resistance in healthy Hong Kong Chinese blood donors–correlation with absence of Arg 506–Gln mutation of Factor V gene. Thromb Haemost 75:522–523PubMedGoogle Scholar
  6. 6.
    Cheung YF, Chay GW, Chiu CS, Cheng LC (2005) Long-term anticoagulation therapy and thromboembolic complications after the Fontan procedure. Int J Cardiol, 19 July 2005; EpubGoogle Scholar
  7. 7.
    Cromme-Dijkhuis AH, Henkens CMA, Bijleveld CMA, et al (1990) Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations. Lancet 336:1087–1090CrossRefPubMedGoogle Scholar
  8. 8.
    Cromme-Dijkhuis AH, Hess J, Hählen K, et al (1993) Specific sequelae after Fontan operation at mid- and long-term follow-up. J Thorac Cardiovasc Surg 106:1126–1132PubMedGoogle Scholar
  9. 9.
    Dobell AR, Trusler GA, Smallhorn JF, Williams WG (1986) Atrial thrombi after the Fontan operation. Ann Thorac Surg 42:664–667CrossRefPubMedGoogle Scholar
  10. 10.
    Driscoll DJ, Offord KP, Feldt RH, et al. (1992) Five- to fifteen-year follow-up after Fontan operation. Circulation 85:469–496CrossRefPubMedGoogle Scholar
  11. 11.
    Fletcher SE, Case CL, Fyfe DA, Gillette PC (1991) Clinical spectrum of venous thrombi in the Fontan patient. Am J Cardiol 68:1721–1722CrossRefPubMedGoogle Scholar
  12. 12.
    Fontan F, Kirklin JW, Fernandez G, et al (1990) Outcome after a “perfect” Fontan operation. Circulation 81:1520–1536CrossRefPubMedGoogle Scholar
  13. 13.
    Jahangiri M, Ross DB, Redington AN, Lincoln C, Shinebourne EA (1994) Thromboembolism after the Fontan procedure and its modifications. Ann Thorac Surg 58:1409–1414CrossRefPubMedGoogle Scholar
  14. 14.
    Jahangiri M, Shore D, Kakkar V, Lincoln C, Shinebourne E (1997) Coagulation factor abnormalities after the Fontan procedure and its modifications. J Thorac Cardiovasc Surg 113:989–993CrossRefPubMedGoogle Scholar
  15. 15.
    Kaulitz R, Luhmer I, Bergmann F, Rodeck B, Hausdorf G (1997) Sequelae after modified Fontan operation: postoperative haemodynamic data and organ function. Heart 78:154–159CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Koster T, Blann AD, Briet E, Vandenbroucke JP, Rosendaal FR (1995) Role of clotting factor VIII in effect of von Willebrand factor on occurrence of deep-vein thrombosis. Lancet 345:152–155CrossRefPubMedGoogle Scholar
  17. 17.
    Lao TT, Yuen PM, Yin JA (1989) Protein S and protein C levels in Chinese women during pregnancy, delivery and the puerperium. Br J Obstet Gynaecol 96:167–170CrossRefPubMedGoogle Scholar
  18. 18.
    Manning PB, Mayer JE, Wernovsky G, Fishberger SB, Walsch EP (1996) Staged operation to Fontan increased the incidence of sinoatrial node dysfunction. J Thorac Cardiovasc Surg 111:833–840CrossRefPubMedGoogle Scholar
  19. 19.
    Monagle P, Cochrane A, McCrindle B, et al. (1998) Thromboembolic complications after Fontan procedures — the role of prophylactic anticoagulation. J Thorac Cardiovasc Surg 115:493–498CrossRefPubMedGoogle Scholar
  20. 20.
    Odegard KC, McGowan FX, Zurakowski D, et al. (2002) Coagulation factor abnormalities following the Fontan procedure: factor VIII is increased and may predispose to thrombosis. J Am Coll Cardiol 106:II-523Google Scholar
  21. 21.
    Rauch R, Ries M, Hofbeck M, et al. (2000) Hemostatic changes following the modified Fontan operation (total cavopulmonary connection). Thromb Haemost 83:678–682PubMedGoogle Scholar
  22. 22.
    Ravn HB, Hjortdal VE, Stenbog EV, et al. (2001) Increased platelet reactivity and significant changes in coagulation markers after cavopulmonary connection. Heart 85:61–65CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    van Nieuwenhuizen RC, Peters M, Lubbers LJ, et al. (1999) Abnormalities in liver function and coagulation profile following the Fontan procedure. Heart 82:40–46CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Wright EM, Royston P (1999) Calculating reference intervals for laboratory measurements. Stat Methods Med Res 8:93–112CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, Inc. 2005

Authors and Affiliations

  1. 1.Division of Paediatric Cardiology, Department of Paediatrics and Adolescent MedicineGrantham Hospital,The University of Hong KongAberdeenPeople’s Republic of China
  2. 2.Division of Haematology, Department of PathologyQueen Mary Hospital, The University of Hong KongPeople’s Republic of China

Personalised recommendations