title: Mid- to long-term results of the two-stage approach for type B interrupted aortic arch and ventricular septal defect.
abstract: BACKGROUND
  Type B interrupted aortic arch with ventricular septal defect is a complex congenital heart defect that may have associated left ventricular outflow tract obstruction. Surgical management has evolved from a two-stage approach to the currently favored single-stage approach. The following data summarize our experience with the two-stage approach over a 15-year period.
  
  
  METHODS
  Between 1980 and 1995, 27 consecutive patients with type B interrupted aortic arch and ventricular septal defect underwent surgical management using the two-stage approach. There were 15 girls and 12 boys; 21 patients had the DiGeorge syndrome.
  
  
  RESULTS
  Stage I was performed at a median age of 4 days. Twenty-six (96%) of 27 patients survived first-stage palliation. One patient survived stage I palliation but died before undergoing stage II. Twenty-five patients underwent second-stage repair at a median age of 6 weeks (range, 1 to 46 weeks). There were 2 early deaths and 1 late death. Actuarial analysis demonstrates 1- and 5-year survival rates of 85% and 81%, respectively. Twenty-two survivors have been followed up for an average of 8 +/- 2 years. Freedom from reoperation for arch graft enlargement has been 86% at 3 years and 55% at 5 years. Freedom from reoperation for left ventricular outflow tract obstruction has been 82% at both 3 and 5 years.
  
  
  CONCLUSIONS
  The two-stage approach can achieve good mid- to long-term palliation of patients with type B interrupted aortic arch and ventricular septal defect. These results should provide a reference from which to gauge the long-term success of the single-stage approach.
authors.affiliation: Cardiac Institute, Children's Hospital and Health Center, San Diego, California, USA.
authors.forename: R D
authors.initials: RD
authors.lastname: Mainwaring
authors.forename: J J
authors.initials: JJ
authors.lastname: Lamberti
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities*; D001024:Aortic Valve Stenosis / Q000150:complications; D002648:Child; D002675:Child, Preschool; D004062:DiGeorge Syndrome / Q000150:complications; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008722:Methods; D010166:Palliative Care; D012189:Retrospective Studies; D015996:Survival Rate; D014694:Ventricular Outflow Obstruction / Q000601:surgery