OBJECTIVE: Using age-matched controls, this study prospectively evaluated coagulation factor abnormalities and hemodynamic variables in @entity1 who had undergone the Fontan operation. METHODS: Coagulation factors were assayed in 20 @entity1 (mean age 6.4 +/- 2.9 years), at a mean 3.7 +/- 2.3 years after the Fontan procedure; 24 healthy @entity1 (mean age 6.8 +/- 2.8 years) were assayed as controls. Concentration of factors II, V, VII, @entity797 , IX, X; @entity5582 ; plasminogen; proteins C and S; fibrinogen; serum albumin; and liver enzymes were measured. Normal reference intervals based on the control @entity1 were determined using 95% confidence limits. @entity1 demographic, hemodynamic variables, and elapsed time after the Fontan procedure were evaluated as possible predictors of @entity854 . RESULTS: Concentrations of protein @entity5582 were significantly lower in Fontan @entity1 compared with age-matched controls (P <.01); factor @entity797 was significantly elevated in 6 @entity1 (35%), 2 of whom had a @entity1031 event. A higher superior vena cava pressure was predictive of an elevated factor @entity797 level (P =.003). No other specific hemodynamic variables were predictive of a procoagulant or anticoagulant abnormality. CONCLUSION: Procoagulant and anticoagulant factor levels were significantly lower in @entity1 after the Fontan operation independent of hemodynamic variables peculiar to the Fontan circulation. Increased factor @entity797 level requires further evaluation as a cause of @entity715 in @entity1 with Fontan physiology and may also indicate a subset of these @entity1 in whom anticoagulation is indicated.

Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor @entity797 may predispose to XXXX .

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'Patient'] @entity797 :: ('1351', 'Gene') :: ['VIII'] @entity854 :: ('MESH:D018376', 'Disease') :: ['coagulation abnormalities'] @entity1031 :: ('MESH:D013923', 'Disease') :: ['thromboembolic'] @entity715 :: ('MESH:D013927', 'Disease') :: ['thrombosis'] @entity5582 :: ('462', 'Gene') :: ['ATIII', 'C; factors II, V, VII, X; plasminogen; and ATIII']
@entity715 :: (MESH:D013927,Disease) :: ['thrombosis']
