title: Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study.
abstract: BACKGROUND
  Patients with both interrupted aortic arch (IAA) and truncus arteriosus (TA) have worse outcomes than those with either lesion in isolation. We determined outcomes and associated factors in this rare group.
  
  
  METHODS
  From 1987 to 1997, 50 (11%) of 472 neonates with IAA were identified with TA. Site of aortic arch interruption was distal to the left subclavian artery in 16% and between the left common carotid and subclavian artery in 84%. From the common arterial trunk, the pulmonary arteries arose from a main pulmonary trunk in 46%, common orifice in 22%, and separate orifices in 32%. At presentation, truncal valve stenosis was present in 12% and regurgitation in 22%.
  
  
  RESULTS
  There were 34 deaths, with a single early hazard phase. Overall survival from admission was 44%, 39%, and 31% at 6 months, 1 year, and 10 years, respectively. One patient had primary cardiac transplantation and 4 died without any intervention. The IAA repair alone was performed in 7 patients, with single stage repair of both IAA and TA in 38 patients. Associated factors for overall time-related death include female gender (p < 0.001), type III TA (p < 0.001) and one institution (low-risk; p < 0.001). Results improved somewhat over time (p < 0.001). At 5 years after IAA repair only 28% were alive without arch repair intervention, and at 5 years after TA repair only 18% were alive without conduit reoperation.
  
  
  CONCLUSIONS
  The combination of IAA and TA carries high early mortality, with high risk of reinterventions in survivors. One stage repair of both TA and IAA is the optimal management.
authors.affiliation: The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
authors.forename: Tara
authors.initials: T
authors.lastname: Karamlou
authors.forename: Eugene H
authors.initials: EH
authors.lastname: Blackstone
authors.forename: Ralph S
authors.initials: RS
authors.lastname: Mosca
authors.forename: Gary K
authors.initials: GK
authors.lastname: Lofland
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Caldarone
authors.forename: William G
authors.initials: WG
authors.lastname: Williams
authors.forename: Andrew S
authors.initials: AS
authors.lastname: Mackie
authors.forename: Brian W
authors.initials: BW
authors.lastname: McCrindle
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000601:surgery; D015331:Cohort Studies; D003131:Combined Modality Therapy; D015897:Comorbidity; D003251:Constriction, Pathologic; D004374:Ductus Arteriosus, Patent / Q000188:drug therapy / Q000453:epidemiology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000453:epidemiology / Q000601:surgery; D016027:Heart Transplantation; D006351:Heart Valves / Q000002:abnormalities / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015995:Prevalence; D011446:Prospective Studies; D011453:Prostaglandins / Q000627:therapeutic use; D011651:Pulmonary Artery / Q000002:abnormalities; D012086:Reoperation; D012121:Respiration, Artificial; D012955:Societies, Medical; D016019:Survival Analysis; D015996:Survival Rate; D013903:Thoracic Surgery; D016896:Treatment Outcome; D014339:Truncus Arteriosus, Persistent / Q000188:drug therapy / Q000453:epidemiology* / Q000601:surgery
chemical_list: D011453:Prostaglandins