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Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations
A H Cromme-Dijkhuis1, C M Henkens, C M Bijleveld
1Division of Pediatric Cardiology, University Hospital, Groningen, The Netherlands.
Insights
Patients undergoing Fontan-type operations face a high risk of thromboembolism due to coagulation factor abnormalities, particularly protein C deficiency. This imbalance contributes to thrombotic events years after congenital heart defect correction.
Area of Science:
- Cardiology
- Hematology
- Pediatric Surgery
Background:
- Fontan-type operations are palliative procedures for complex congenital heart defects.
- Patients with corrected congenital heart defects may experience long-term complications, including thromboembolism.
Purpose of the Study:
- To investigate the prevalence of coagulation factor abnormalities in patients post-Fontan-type operation.
- To determine if these abnormalities correlate with the high incidence of thromboembolism.
Main Methods:
- Screening of 37 patients post-Fontan-type operation for coagulation factor abnormalities.
- Analysis of identified abnormalities, focusing on protein C, antithrombin III, and factors II and X.
Main Results:
- 3 out of 37 patients experienced severe thromboembolism.
- 24 out of 37 patients exhibited 63 coagulation factor abnormalities.
- Protein C deficiency was the most common abnormality, with significantly lower levels of antithrombin III, factors II, and X in affected patients.
Conclusions:
- Coagulation factor abnormalities, especially protein C deficiency, are prevalent in patients after Fontan-type operations.
- An imbalance between procoagulant and anticoagulant factors likely contributes to the elevated thrombotic risk in this population.
Abstract:
Severe thromboembolism occurred in 3 of 37 patients who had undergone Fontan-type operations for correction of congenital heart defects several months to years after the operation. The patients were screened for coagulation factor abnormalities to find out whether known prothrombotic risk factors could explain the high frequency of thromboembolism. 63 abnormalities were found in 24 of the 37 patients. The commonest and most pronounced abnormality was deficiency of protein C, a known risk factor. Concentrations of antithrombin III and factors II and X were significantly lower in protein-C-deficient patients than in those with normal protein C concentrations. These findings suggest that the high thrombotic risk in these patients is caused or at least associated with an imbalance between procoagulant and anticoagulant factors.