title: Clinical and Functional Developmental Outcomes in Neonates Undergoing Truncus Arteriosus Repair: A Cohort Study.
abstract: BACKGROUND
  Truncus arteriosus (TA) is an uncommon congenital cardiac lesion that portends an exceedingly poor prognosis if not repaired. The objective of this study was to assess the clinical and developmental outcomes in a prospective cohort of patients who underwent TA repair.
  
  
  METHODS
  All patients who underwent a TA repair between 1996 and 2012 were included. Follow-up clinical, neurologic, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Functional developmental outcomes were assessed at 21.1 ± 2.5 months of age with the Adaptive Behavior Assessment System-II, General Adaptive Composite (GAC) score. Survival and outcomes were compared between those with and without chromosomal abnormalities (CA). Survival and freedom from reintervention were assessed by Kaplan-Meier analysis.
  
  
  RESULTS
  The study comprised 36 infants (19 male). CA was identified in 13, with 22q11.2 deletion in 10 patients. Patients underwent TA repair at a median age of 10 days; 5 patients underwent concomitant interrupted arch repair. There were 8 deaths, 2 of which occurred in the hospital. The 5-year survival was 79.4%. Survival was similar between those with and without CA. At 5 years, freedom from reoperation was 77.2%. The mean GAC was higher in the patients without CA (93.6 ± 12.8 vs 76.1 ± 13.1, p = 0.0016).
  
  
  CONCLUSIONS
  Patients with surgically repaired TA continue to have significant postoperative mortality. Reoperation and cardiac catheterization are eventualities for a quarter of patients in the first 5 years of life. Functional developmental outcome in patients without CA is good, although it is significantly impaired in those with CA.
authors.affiliation: Department of Surgery, University of Alberta, Edmonton, Alberta, Canada. Electronic address: billiejean@ualberta.ca.
authors.forename: Billie-Jean
authors.initials: BJ
authors.lastname: Martin
authors.affiliation: Department of Surgery, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: David B
authors.initials: DB
authors.lastname: Ross
authors.affiliation: Pediatric Rehabilitation Outcomes Unit, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada; Pediatric Intensive Care, Stollery Children's Hospital, Edmonton, Alberta, Canada.
authors.forename: Gwen Y
authors.initials: GY
authors.lastname: Alton
authors.affiliation: Pediatric Intensive Care, Stollery Children's Hospital, Edmonton, Alberta, Canada; Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Ari R
authors.initials: AR
authors.lastname: Joffe
authors.affiliation: Pediatric Rehabilitation Outcomes Unit, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada; Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Charlene M T
authors.initials: CM
authors.lastname: Robertson
authors.affiliation: Department of Surgery, University of Alberta, Edmonton, Alberta, Canada; Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Ivan M
authors.initials: IM
authors.lastname: Rebeyka
authors.affiliation: Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Joseph
authors.initials: J
authors.lastname: Atallah
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology; D001013:Aorta, Thoracic / Q000002:abnormalities / Q000601:surgery; D025063:Chromosome Disorders / Q000453:epidemiology; D015897:Comorbidity; D004363:Drug Utilization; D005260:Female; D005500:Follow-Up Studies; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D065886:Neurodevelopmental Disorders / Q000453:epidemiology* / Q000209:etiology; D011183:Postoperative Complications / Q000401:mortality; D011379:Prognosis; D011446:Prospective Studies; D012086:Reoperation / Q000706:statistics & numerical data; D016896:Treatment Outcome; D014339:Truncus Arteriosus, Persistent / Q000601:surgery*