title: Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure.
abstract: OBJECTIVE
  Thromboembolic events in patients who have undergone the Fontan operation have been reported to be as high as 20% to 33%. A hypercoagulable state with deficiencies in proteins C and S has been implicated. Using age-matched control subjects, we evaluated whether an altered coagulation state is present earlier in the course of staged single-ventricle repair.
  
  
  METHODS
  After informed consent had been obtained, coagulation factors were assayed in 36 infants (mean age, 7.7 +/- 3.6 months) with single-ventricle cardiac defects immediately before undergoing the bidirectional Glenn procedure; 34 infants (mean age, 8.4 +/- 2.6 months) without cardiac disease were assayed as control subjects. Concentration of factors II, V, VII, VIII, IX, and X; antithrombin III; plasminogen; proteins C and S; fibrinogen; serum albumin; and liver enzymes were measured. Normal reference intervals on the basis of the control subjects were determined by using 95% confidence limits. Patient demographic and hemodynamic variables were evaluated as possible predictors of coagulation abnormalities.
  
  
  RESULTS
  Concentrations of protein C; factors II, V, VII, IX, and X; plasminogen; fibrinogen; and antithrombin III were significantly lower in the pre-Glenn infants compared with the age-matched control subjects (all P <.001, Student t test). On the basis of multiple logistic regression, no specific hemodynamic variables were predictive of a procoagulant or anticoagulant deficiency. Ventricular dysfunction did predict the presence of multiple coagulation abnormalities (P <.001).
  
  
  CONCLUSIONS
  Procoagulant and anticoagulant factor abnormalities occur early in the course of single-ventricle repair and precede the cavopulmonary connection.
authors.affiliation: Department of Anesthesia, Division of Hematology, Children's Hospital, 300 Longwood Avenue, Boston, MA 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: James A
authors.initials: JA
authors.lastname: DiNardo
authors.forename: Robert A
authors.initials: RA
authors.lastname: Castro
authors.forename: David
authors.initials: D
authors.lastname: Zurakowski
authors.forename: Caroline M
authors.initials: CM
authors.lastname: Connor
authors.forename: Dolly D
authors.initials: DD
authors.lastname: Hansen
authors.forename: Ellis J
authors.initials: EJ
authors.lastname: Neufeld
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; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000150:complications / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities*; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D016015:Logistic Models; D008297:Male; D012016:Reference Values; D019851:Thrombophilia / Q000097:blood* / Q000150:complications*
chemical_list: D001779:Blood Coagulation Factors