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Extracardiac conduit Fontan for children with heterotaxy and functionally single ventricle
1Cardiac Institute, Children's Hospital and Health Center, San Diego, CA, USA.
Insights
Extracardiac conduits effectively complete Fontan circulation in children with single ventricle and visceral heterotaxy. This surgical approach offers a successful treatment option with good outcomes and no thromboembolic complications.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Medical Devices
Background:
- Visceral heterotaxy with a functionally single ventricle presents unique Fontan completion challenges due to abnormal venous drainage.
- An extracardiac conduit is a surgical option to reroute systemic venous blood to pulmonary arteries.
Purpose of the Study:
- To evaluate the efficacy and safety of extracardiac conduits for Fontan completion in children with visceral heterotaxy and a functionally single ventricle.
Main Methods:
- A retrospective review of nine children with heterotaxy and a single ventricle who underwent extracardiac conduit placement.
- All patients had a prior bidirectional Glenn procedure; extracardiac conduits were synthetic (Gore-Tex).
Main Results:
- Six of nine children had an uneventful recovery; one required a fenestration procedure for elevated venous pressures.
- Two patients developed pleural effusions; median hospital stay was 10 days.
- All children survived with a mean follow-up of 19 months; no thromboembolic events occurred.
Conclusions:
- Extracardiac conduits are a well-functioning option for Fontan circulation completion in this complex pediatric population.
- This technique provides a successful surgical solution for children with single ventricle physiology and visceral heterotaxy.
Unlabelled:
Children with functionally single ventricle in the setting of visceral heterotaxy (isomerism) may present a surgical challenge at the time of Fontan completion because of anomalies of systemic and pulmonary venous drainage. We have used an extracardiac conduit in this population to direct inferior caval venous blood to the pulmonary arteries. Over the past five years, nine children with heterotaxy and a functionally single ventricle underwent correction by placement of an extracardiac synthetic (Gore-Tex) conduit. All patients had previously undergone a bidirectional Glenn procedure. Age and weight at the time of insertion of the extracardiac conduit were 26 +/- 15 months, and 11 +/- 2 kilograms, respectively.
Results:
Of the nine children, six had an uneventful recovery. One developed elevated venous pressures and required a 'fenestration procedure'. Two patients developed pleural effusions. Median length of stay in hospital was 10 days. All children are alive and well, with follow-up of 19 +/- 16 months. There have been no thromboembolic complications.
Conclusions:
The extracardiac conduit has worked well in our experience for the completion of the Fontan circulation in children with functionally single ventricle in the setting of visceral heterotaxy.
