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Coagulation factor abnormalities in patients with single-ventricle physiology immediately prior to the Fontan
Kirsten C Odegard1, Francis X McGowan, David Zurakowski
1Department of Anesthesia, Children's Hospital, and Harvard Medical School, Boston, Massachusetts 02115, USA. kirsten.odegard@tch.harvard.edu
Insights
Coagulation abnormalities are present in children before the Fontan operation, indicating risks for bleeding or clotting early in single-ventricle repair. These findings highlight the need for monitoring coagulation status in pediatric cardiac surgery patients.
Area of Science:
- Pediatric Cardiology
- Hemostasis and Thrombosis
- Congenital Heart Disease
Background:
- Coagulation abnormalities are a known complication following the Fontan operation.
- These abnormalities are often attributed to the unique physiology associated with the Fontan procedure.
- This study investigates whether these coagulation issues are present even before the Fontan operation.
Purpose of the Study:
- To evaluate coagulation abnormalities in children undergoing a bidirectional Glenn procedure before the Fontan operation.
- To test the hypothesis that coagulation abnormalities exist prior to the Fontan procedure.
- To identify potential predictors of these abnormalities.
Main Methods:
- Assayed coagulation factors in 38 children before the Fontan operation and compared them to 37 healthy controls.
- Measured concentrations of specific coagulation factors (II, V, VII, X, etc.), antithrombin III, plasminogen, proteins C and S, fibrinogen, albumin, and liver enzymes.
- Analyzed demographic data, hemodynamic variables, and time elapsed since the Glenn procedure.
Main Results:
- Children had significantly lower concentrations of protein C, factors II, V, VII, X, plasminogen, and antithrombin III compared to controls (p < 0.01).
- No hemodynamic variables predicted anticoagulant or procoagulant deficiencies.
- Longer intervals between the bidirectional Glenn and Fontan procedures correlated with lower levels of factor VII, protein S, and protein C (p < 0.001).
Conclusions:
- Coagulation abnormalities are evident early in the staged single-ventricle repair process.
- These early abnormalities may predispose patients to an increased risk of clotting or bleeding.
- Monitoring coagulation status is crucial in patients undergoing staged single-ventricle palliation.
Background:
Coagulation abnormalities have been reported following the Fontan operation and have been attributed to various aspects of Fontan-associated physiology. Using age-matched controls, this study evaluated coagulation abnormalities in children who had undergone a bidirectional Glenn procedure to test the hypothesis that coagulation abnormalities are present before the Fontan operation.
Methods:
Coagulation factors were assayed in 38 children (mean age 34.4 +/- 15 months) immediately before the Fontan operation; 37 healthy children (mean age 33 +/- 17 months) were assayed as controls. Concentration of factors II, V, VII, VIII, IX, and X and of antithrombin III, 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 data, hemodynamic variables, and elapsed time after the Glenn procedure were evaluated as possible predictors of coagulation abnormalities.
Results:
Concentrations of protein C; factors II, V, VII, and X; plasminogen; and antithrombin III were significantly lower before the Fontan operation compared with age-matched controls (p < 0.01); no specific hemodynamic variables were predictive of a pro- or anticoagulant deficiency. There were significant positive correlations between patients who had abnormally low factor VII, protein S, and protein C levels and a longer interval between the bidirectional Glenn procedure and the Fontan operation (p < 0.001).
Conclusions:
Coagulation abnormalities that could predispose patients to increased risk for clotting or bleeding are evident early in the course of staged single-ventricle repair.