EXTRACARDIAC CONDUIT FONTAN FOR CHILDREN WITH HETEROTAXY AND FUNCTIONALLY SINGLE VENTRICLE

Children with functionally single ventricle in the setting of visceral heterotaxy (isomerism) may present a surgical challenge at the time o f Fontan completion because of anomalies of systemic and pulmonary ven ous 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 r ecovery. One developed elevated venous pressures and required a 'fenes tration procedure'. Two patients developed pleural effusions. Median l ength 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 i n our experience for the completion of the Fontan circulation in child ren with functionally single ventricle in the setting of visceral hete rotaxy.