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Prospective longitudinal study of coagulation profiles in children with hypoplastic left heart syndrome from stage I
Kirsten C Odegard1, David Zurakowski, James A DiNardo
1Department of Anesthesiology, Perioperative and Pain Medicine, Children's Hospital Boston, Boston, MA 02115, USA. Kirsten.odegard@childrens.harvard.edu
Insights
Children undergoing the Fontan operation experience acquired coagulation defects, notably increased factor VIII levels post-procedure. Monitoring factor VIII is crucial for identifying thrombosis risk in these patients.
Area of Science:
- Pediatric Cardiology
- Hematology
- Congenital Heart Disease
Background:
- The Fontan operation increases thrombosis risk in patients with univentricular heart disease.
- It remains unclear if coagulation anomalies are intrinsic or acquired during staged repair.
Purpose of the Study:
- To prospectively evaluate changes in coagulation profiles in hypoplastic left heart syndrome patients.
- To determine if coagulation defects are acquired during staged surgical palliation leading to the Fontan operation.
Main Methods:
- Prospective longitudinal study of 37 hypoplastic left heart syndrome patients.
- Coagulation factors (II, V, VII, VIII, IX, X, proteins C & S, fibrinogen, antithrombin) and albumin measured at multiple surgical stages.
- Comparison with age-matched healthy controls; evaluation of demographic and hemodynamic predictors.
Main Results:
- Significantly lower levels of procoagulation and anticoagulation factors were observed throughout the study period.
- A significant increase in factor VIII levels was noted after the Fontan procedure.
- No correlation was found between factor VIII levels and hemodynamic variables or liver function.
Conclusions:
- The study confirms an acquired defect, specifically elevated factor VIII levels, after the Fontan operation.
- This acquired defect warrants further investigation into its cause.
- Monitoring factor VIII levels post-Fontan may identify patients at increased risk for thrombosis.
Objective:
The risk for thrombosis is increased after the Fontan operation. It is unknown whether children with univentricular heart disease have an intrinsic coagulation anomaly or acquire a defect in coagulation during the course of the staged repair. This prospective, longitudinal study evaluated changes in coagulation profiles in a cohort of patients with hypoplastic left heart syndrome from stage I palliation through completion of the Fontan operation.
Methods:
Thirty-seven patients with hypoplastic left heart syndrome were enrolled prospectively, and the concentration of factors II, V, VII, VIII, IX, X, proteins C and S, fibrinogen, antithrombin, serum albumin, and liver enzymes were measured before stage I palliation (mean age 4 +/- 2 days), before bidirectional Glenn (mean age 5.9 +/- 1.8 months), before the Fontan procedure (mean age 27.1 +/- 6.6 months), and after the Fontan procedure (mean age 49 +/- 17.6 months). Healthy children were used as age-matched controls for coagulation factors. Demographic, hemodynamic variables, and elapsed time after the Fontan procedure were evaluated as possible predictors of coagulation abnormalities.
Results:
Significantly lower levels of both procoagulation and anticoagulation factors were demonstrated through to completion of the Fontan procedure. After the Fontan procedure, there was a significantly higher factor VIII level (P < .005) but no correlation with hemodynamic variables or liver function.
Conclusion:
This longitudinal study in patients with identical cardiac disease and staged surgical procedures confirms the increase in factor VIII level after the Fontan procedure. This is an acquired defect, and although the cause remains to be determined, monitoring factor VIII levels after the Fontan operation could indicate a subset of patients at risk for thrombosis.