title: Surgical repair of truncus arteriosus associated with interrupted aortic arch: long-term outcomes.
abstract: BACKGROUND
  Truncus arteriosus (TA) with interrupted aortic arch (IAA) is a rare combination of complex anomalies. We report long-term follow-up after one-stage repair of TA associated with IAA.
  
  
  METHODS
  From April 1985 to August 2007, 16 patients with TA associated with IAA underwent one-stage repair.
  
  
  RESULTS
  There were 2 (12.5%) early deaths and no late deaths. Follow-up was 92.9% complete (13 of 14 patients), with 1 patient lost to follow-up. Median duration of follow-up was 18.2 years (range, 2.1 to 21.9 years). Functional status in all patients was good. Thirteen patients underwent 25 surgical reoperations and 5 interventional procedures (3 aortic arch balloon angioplasties and 2 pulmonary artery balloon angioplasties). Overall freedom from any reoperation was 69.2% at 1 month, 54.5% at 3 years, 30% at 5 years, 11.1% at 10 years, and 0% at 15 years after the initial operation. Freedom from aortic reoperation was 76.9% at 1 month, 72.7% at 3 years, 70% at 5 years, 66.7% at 10 years, and 57.1% at 15 years; while freedom from right ventricular-to-pulmonary artery conduit replacement was 84.6% at 1 month, 63.6% at 3 years, 40% at 5 years, 11.1% at 10 years, and 0% at 15 years. Finally, freedom from truncal valve reoperation was 100% at 5 years, 88.9% at 10 years, and 85.7% at 15 years.
  
  
  CONCLUSIONS
  One-stage repair of TA-IAA can be undertaken with good long-term results. Despite a significant reoperation rate, patients' functional status remains good at the long-term follow-up.
authors.affiliation: Cardiac Surgery Unit, Royal Children's Hospital, Melbourne, Australia.
authors.forename: Lyubomyr
authors.initials: L
authors.lastname: Bohuta
authors.forename: Aisyah
authors.initials: A
authors.lastname: Hussein
authors.forename: Tyson A
authors.initials: TA
authors.lastname: Fricke
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
authors.forename: Martin
authors.initials: M
authors.lastname: Bennett
authors.forename: Christian
authors.initials: C
authors.lastname: Brizard
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000601:surgery; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality*; D002315:Cardiopulmonary Bypass; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D017052:Hospital Mortality / Q000639:trends*; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000401:mortality; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D056346:Sternotomy / Q000379:methods; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome; D014339:Truncus Arteriosus, Persistent / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*