title: Hemostatic changes following the modified Fontan operation (total cavopulmonary connection).
abstract: 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.
authors.affiliation: Children's Hospital of the Friedrich-Alexander-Universität, Erlangen-Nuernberg, Germany. ralf.rauch@dland.de
authors.forename: R
authors.initials: R
authors.lastname: Rauch
authors.forename: M
authors.initials: M
authors.lastname: Ries
authors.forename: M
authors.initials: M
authors.lastname: Hofbeck
authors.forename: G
authors.initials: G
authors.lastname: Buheitel
authors.forename: H
authors.initials: H
authors.lastname: Singer
authors.forename: J
authors.initials: J
authors.lastname: Klinge
mesh_terms: D000293:Adolescent; D000328:Adult; D001779:Blood Coagulation Factors / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004789:Enzyme Activation; D005260:Female; D005342:Fibrinolysis; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000097:blood / Q000601:surgery; D006429:Hemiplegia / Q000209:etiology; D006487:Hemostasis; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000209:etiology; D011486:Protein C / Q000032:analysis; D017293:Protein S / Q000032:analysis; D013917:Thrombin / Q000378:metabolism; D019851:Thrombophilia / Q000209:etiology*; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D001779:Blood Coagulation Factors; D011486:Protein C; D017293:Protein S; D005723:gamma-Glutamyltransferase; D013917:Thrombin