Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure.
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.
Odegard
Kirsten C
KC
Department of Anesthesia, Division of Hematology, Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA. kirsten.odegard@tch.harvard.edu
McGowan
Francis X
FX
DiNardo
James A
JA
Castro
Robert A
RA
Zurakowski
David
D
Connor
Caroline M
CM
Hansen
Dolly D
DD
Neufeld
Ellis J
EJ
del Nido
Pedro J
PJ
Laussen
Peter C
PC
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*
D001779:Blood Coagulation Factors