title: Long-term outcomes of single-ventricle palliation for unbalanced atrioventricular septal defects: Fontan survivors do better than previously thought.
abstract: Single-ventricle palliation (SVP) for children with unbalanced atrioventricular septal defect (uAVSD) is thought to carry a poor prognosis, but limited data have been reported.
  
  
  
  We performed a retrospective review of children with uAVSD who underwent SVP at a single institution. Data were obtained from medical records and correspondence with general practitioners and cardiologists.
  
  
  
  Between 1976 and 2016, a total of 139 patients underwent SVP for uAVSD. A neonatal palliative procedure was performed in 83.5% of these patients (116 of 139), and early mortality occurred in 11.2% (13 of 116). Ninety-four patients underwent stage II palliation, with an early mortality of 6.4% (6 of 94). Eighty patients (57.6%) underwent Fontan completion, with an early mortality of 3.8% (3 of 80). Interstage mortality was 11.7% (12 of 103) between stages I and II and 17.0% (15 of 88) between stage II and Fontan. Long-term survival was 66.5% (95% confidence interval [CI], 57.9%-73.9%) at 5 years, 64.4% (95% CI, 55.5%-72.0%) at 15 years, and 57.8% (95% CI, 47.5%-66.8%) at 25 years. Survival post-Fontan was 94.9% (95% CI, 86.9%-98.0%) at 5 years, 92.0% (95% CI, 80.6%-96.8%) at 15 years, and 82.4% (95% CI, 61.5%-92.6%) at 25 years. Risk factors associated with death or transplantation were aortic atresia (hazard ratio [HR], 5.3; P = .03) and hypoplastic aortic arch (HR, 2.5; P = .02). Atrioventricular valve operations were required in 31.7% of the patients (44 of 139), with 31.8% of them (14 of 44) requiring a further operation.
  
  
  
  Children undergoing SVP for uAVSD have substantial mortality, with <60% survival at 25 years. However, survival of children who achieve Fontan completion is better than has been reported previously.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Edward
authors.initials: E
authors.lastname: Buratto
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Xin Tao
authors.initials: XT
authors.lastname: Ye
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Gregory
authors.initials: G
authors.lastname: King
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: William Y
authors.initials: WY
authors.lastname: Shi
authors.affiliation: Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia; Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. Electronic address: igor.konstantinov@rch.org.au.
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
mesh_terms: D000015:Abnormalities, Multiple; D000293:Adolescent; D001315:Australia / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D005544:Forecasting; D006343:Heart Septal Defects / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D010166:Palliative Care; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome; D055815:Young Adult
keywords: Fontan; single-ventricle palliation; unbalanced atrioventricular septal defect