Hemostatic changes following the modified Fontan operation (total cavopulmonary connection)

Thromb Haemost. 2000 May;83(5):678-82.


Thromboembolism is a serious complication after Fontan operation, which may be caused by alterations of the coagulation system. We therefore investigated pro- and anticoagulant factors in 20 patients aged 4 to 21 years, 4 to 63 months following total cavopulmonary connection. Furthermore we compared markers of thrombin activation and fibrinolysis and in vitro clotting and clot-lysis to age-matched healthy subjects. Compared to results of age-matched controls, the Fontan operated individuals had significant decreases in levels of protein C (0.88 U/ml in controls, 0.67 U/ml in patients; p <0.001) and protein S (1.05 in controls, 0.93 U/ml in patients; p <0.05). Moreover, half of the patients had high values of FVIII (>1.5 IU/ml), which are associated with an increased thrombotic risk. These changes may result in enhanced generation of thrombin and plasmin, indicated by our finding of increased thrombin-antithrombin III (TAT) and plasmin-antiplasmin (PAP) levels and a similar trend in prothrombin fragments F1+2. Clot lysis tests, global coagulation tests, red blood cell count, liver enzymes AST, ALT, but not GGT, were generally within the normal ranges.

MeSH terms

  • Adolescent
  • Adult
  • Blood Coagulation Factors / analysis
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Enzyme Activation
  • Female
  • Fibrinolysis
  • Fontan Procedure / adverse effects*
  • Heart Defects, Congenital / blood
  • Heart Defects, Congenital / surgery
  • Hemiplegia / etiology
  • Hemostasis*
  • Humans
  • Male
  • Postoperative Complications / etiology
  • Protein C / analysis
  • Protein S / analysis
  • Thrombin / metabolism
  • Thrombophilia / etiology*
  • gamma-Glutamyltransferase / blood


  • Blood Coagulation Factors
  • Protein C
  • Protein S
  • gamma-Glutamyltransferase
  • Thrombin