title: Coagulation factor abnormalities in patients with single-ventricle physiology immediately prior to the Fontan procedure.
abstract: BACKGROUND
  Coagulation abnormalities have been reported following the Fontan operation and have been attributed to various aspects of Fontan-associated physiology. Using age-matched controls, this study evaluated coagulation abnormalities in children who had undergone a bidirectional Glenn procedure to test the hypothesis that coagulation abnormalities are present before the Fontan operation.
  
  
  METHODS
  Coagulation factors were assayed in 38 children (mean age 34.4 +/- 15 months) immediately before the Fontan operation; 37 healthy children (mean age 33 +/- 17 months) were assayed as controls. Concentration of factors II, V, VII, VIII, IX, and X and of antithrombin III, plasminogen, proteins C and S, fibrinogen, serum albumin, and liver enzymes were measured. Normal reference intervals based on the control patients were determined using 95% confidence limits. Patient demographic data, hemodynamic variables, and elapsed time after the Glenn procedure were evaluated as possible predictors of coagulation abnormalities.
  
  
  RESULTS
  Concentrations of protein C; factors II, V, VII, and X; plasminogen; and antithrombin III were significantly lower before the Fontan operation compared with age-matched controls (p < 0.01); no specific hemodynamic variables were predictive of a pro- or anticoagulant deficiency. There were significant positive correlations between patients who had abnormally low factor VII, protein S, and protein C levels and a longer interval between the bidirectional Glenn procedure and the Fontan operation (p < 0.001).
  
  
  CONCLUSIONS
  Coagulation abnormalities that could predispose patients to increased risk for clotting or bleeding are evident early in the course of staged single-ventricle repair.
authors.affiliation: Department of Anesthesia, Children's Hospital, and Harvard Medical School, Boston, Massachusetts 02115, USA. kirsten.odegard@tch.harvard.edu
authors.forename: Kirsten C
authors.initials: KC
authors.lastname: Odegard
authors.forename: Francis X
authors.initials: FX
authors.lastname: McGowan
authors.forename: David
authors.initials: D
authors.lastname: Zurakowski
authors.forename: James A
authors.initials: JA
authors.lastname: DiNardo
authors.forename: Robert A
authors.initials: RA
authors.lastname: Castro
authors.forename: Pedro J
authors.initials: PJ
authors.lastname: del Nido
authors.forename: Peter C
authors.initials: PC
authors.lastname: Laussen
mesh_terms: D001779:Blood Coagulation Factors / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D006345:Heart Septal Defects, Ventricular / Q000097:blood*; D006801:Humans; D007223:Infant; D008297:Male; D011300:Preoperative Care; D011446:Prospective Studies
chemical_list: D001779:Blood Coagulation Factors