title: Extracardiac conduit Fontan for children with heterotaxy and functionally single ventricle.
abstract: 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.
authors.affiliation: Cardiac Institute, Children's Hospital and Health Center, San Diego, CA, USA.
authors.forename: R D
authors.initials: RD
authors.lastname: Mainwaring
authors.forename: J J
authors.initials: JJ
authors.lastname: Lamberti
mesh_terms: D000015:Abnormalities, Multiple; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000379:methods*; D006801:Humans; D007223:Infant; D007902:Length of Stay; D008297:Male; D011859:Radiography; D016896:Treatment Outcome; D018754:Ventricular Dysfunction / Q000000981:diagnostic imaging / Q000601:surgery*; D014781:Viscera / Q000002:abnormalities*