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Published on: September 9, 2012
Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor VIII may
Kirsten C Odegard1, Francis X McGowan, David Zurakowski
1Department of Anesthesia, Children's Hospital, Boston, MA 02115, USA. kirsten.odegard@tch.harvard.edu
Insights
Children after the Fontan operation show lower levels of key coagulation factors. Elevated factor VIII in some patients warrants further investigation for thrombosis risk and potential anticoagulation therapy.
Area of Science:
- Pediatric Cardiology
- Hematology
- Coagulation Science
Background:
- The Fontan operation is a palliative procedure for complex congenital heart disease.
- Coagulation abnormalities are a known complication in Fontan patients.
- Hemodynamic changes post-Fontan may influence coagulation factor levels.
Purpose of the Study:
- To prospectively evaluate coagulation factor abnormalities in children post-Fontan operation.
- To assess the relationship between hemodynamic variables and coagulation status.
- To identify potential predictors of coagulation abnormalities in this population.
Main Methods:
- Assayed coagulation factors in 20 children post-Fontan and 24 age-matched controls.
- Measured concentrations of multiple coagulation factors, inhibitors, and liver enzymes.
- Evaluated demographic, hemodynamic, and time-related variables as predictors.
Main Results:
- Fontan patients exhibited significantly lower levels of protein C, factors II, V, VII, X, plasminogen, and ATIII compared to controls.
- Factor VIII was elevated in 35% of Fontan patients, with two experiencing thromboembolic events.
- Higher superior vena cava pressure predicted elevated factor VIII, but no other hemodynamic factors predicted coagulation abnormalities.
Conclusions:
- Procoagulant and anticoagulant factor levels are generally reduced in Fontan survivors, irrespective of Fontan-specific hemodynamics.
- Elevated factor VIII levels in Fontan patients require further study as a potential cause of thrombosis.
- Identifying patients with elevated factor VIII may guide decisions regarding anticoagulation therapy.
Objective:
Using age-matched controls, this study prospectively evaluated coagulation factor abnormalities and hemodynamic variables in children who had undergone the Fontan operation.
Methods:
Coagulation factors were assayed in 20 children (mean age 6.4 +/- 2.9 years), at a mean 3.7 +/- 2.3 years after the Fontan procedure; 24 healthy children (mean age 6.8 +/- 2.8 years) were assayed as controls. Concentration of factors II, V, VII, VIII, IX, X; ATIII; 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, hemodynamic variables, and elapsed time after the Fontan procedure were evaluated as possible predictors of coagulation abnormalities.
Results:
Concentrations of protein C; factors II, V, VII, X; plasminogen; and ATIII were significantly lower in Fontan patients compared with age-matched controls (P <.01); factor VIII was significantly elevated in 6 patients (35%), 2 of whom had a thromboembolic event. A higher superior vena cava pressure was predictive of an elevated factor VIII 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 patients after the Fontan operation independent of hemodynamic variables peculiar to the Fontan circulation. Increased factor VIII level requires further evaluation as a cause of thrombosis in patients with Fontan physiology and may also indicate a subset of these patients in whom anticoagulation is indicated.
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