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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

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). CONCLUSION: Procoagulant and anticoagulant factor abnormalities occur early in the course of single-ventricle repair and precede the cavopulmonary connection.[1]


  1. Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure. Odegard, K.C., McGowan, F.X., DiNardo, J.A., Castro, R.A., Zurakowski, D., Connor, C.M., Hansen, D.D., Neufeld, E.J., del Nido, P.J., Laussen, P.C. J. Thorac. Cardiovasc. Surg. (2002) [Pubmed]
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