title: Management and outcome of infants and children with right atrial isomerism.
abstract: OBJECTIVE
  To assess the current results and outcome of surgery in infants and children with right atrial isomerism and complex congenital heart disease.
  
  
  METHODS
  Tertiary referral centre.
  
  
  METHODS
  20 consecutive children with right atrial isomerism and complex congenital heart disease underwent surgery over a 6 year period between August 1987 and July 1993. The results and outcome were analysed according to age, presentation, and surgical procedures.
  
  
  RESULTS
  Patients were divided into two groups depending on age at presentation and initial surgery: group A comprised 11 patients who required surgical intervention in the first month of life (mean age 5 days); and group B comprised nine patients who required initial surgical intervention after the first month of life (mean age 6.8 months). Seven (64%) of the 11 patients in group A had obstructed pulmonary venous drainage and ten (91%) had pulmonary atresia. There were seven early deaths (64%), including the five patients who required systemic to pulmonary artery shunt and simultaneous repair of obstructed pulmonary veins. The long-term survival rate in this group was 18% (two of 11). Pulmonary venous obstruction was present in two (22%) of the nine patients in group B and four (44%) had pulmonary atresia. There were no early deaths. One patient died after a second palliative procedure. There was one late sudden death. Four patients had a Fontan operation with no deaths. Two of the remaining three patients meet the Fontan criteria. The long-term survival rate in this group was 78% (seven of nine).
  
  
  CONCLUSIONS
  Surgical management of patients with right atrial isomerism who have complex congenital heart disease carries a high mortality and remains palliative. The overall survival rate was 45% (nine of 20); 18% in patients requiring surgery in the first month of life (group A) and 78% in patients requiring surgery after the first month of life (group B) (P < 0.001). Of the total of 20 patients, nine were potential candidates for a Fontan operation. Seven of these have undergone a Fontan procedure with five survivors.
authors.affiliation: Heart Unit, Children's Hospital, Birmingham.
authors.forename: M
authors.initials: M
authors.lastname: Sadiq
authors.forename: O
authors.initials: O
authors.lastname: Stümper
authors.forename: J V
authors.initials: JV
authors.lastname: De Giovanni
authors.forename: J G
authors.initials: JG
authors.lastname: Wright
authors.forename: B
authors.initials: B
authors.lastname: Sethia
authors.forename: W J
authors.initials: WJ
authors.lastname: Brawn
authors.forename: E D
authors.initials: ED
authors.lastname: Silove
mesh_terms: D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D006325:Heart Atria / Q000002:abnormalities* / Q000601:surgery; D006330:Heart Defects, Congenital / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008485:Medical Audit; D015996:Survival Rate; D016896:Treatment Outcome