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Single-ventricle palliation in children with atrioventricular septal defect and transposition of the great arteries: 45 years of experience

Published online by Cambridge University Press:  29 June 2020

Edward Buratto Open the ORCID record for Edward Buratto [Opens in a new window] ,
Tyson A. Fricke ,
Xin Tao Ye ,
Johann Brink ,
Christian P. Brizard ,
Yves d’Udekem  and
Igor E. Konstantinov
Edward Buratto
Affiliation:
Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Murdoch Children’s Research Institute, Melbourne, Australia
Tyson A. Fricke
Affiliation:
Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Murdoch Children’s Research Institute, Melbourne, Australia
Xin Tao Ye
Affiliation:
Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Murdoch Children’s Research Institute, Melbourne, Australia
Johann Brink
Affiliation:
Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Murdoch Children’s Research Institute, Melbourne, Australia
Christian P. Brizard
Affiliation:
Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Murdoch Children’s Research Institute, Melbourne, Australia
Yves d’Udekem
Affiliation:
Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Murdoch Children’s Research Institute, Melbourne, Australia
Igor E. Konstantinov*
Affiliation:
Department of Cardiac Surgery, The Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Murdoch Children’s Research Institute, Melbourne, Australia Melbourne Children’s Centre for Cardiovascular Genomics and Regenerative Medicine, Melbourne, Australia
*
Author for correspondence: Professor Igor E. Konstantinov, Royal Children’s Hospital, Flemington Road, Parkville, VIC3029, Australia. Tel: +61 3 9345 5200; Fax: +61 3 9345 6386. E-mail: igor.konstantinov@rch.org.au
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Abstract

Background:

The association of atrioventricular septal defect and transposition of the great arteries is very rare. As a rule, these patients have unbalanced ventricles. However, there have been no studies describing the results of single-ventricle palliation in these children.

Methods:

All children who underwent surgery with a diagnosis of atrioventricular septal defect and transposition of the great arteries were included in the study. Data were obtained from medical records.

Results:

A total of 38 patients with atrioventricular septal defect and transposition of the great arteries underwent single-ventricle palliation at the study institution between 1971 and 2016. The mean follow-up was 12.4 years (median: 14.6 years, range 2–43.3 years). Most children had unbalanced atrioventricular septal defect (94.7%, 36/38). Survival was 67.6% (95% confidence interval [CI]: 50.0–80.2%) at 10 years and 57.8% (95% CI: 38.0–73.4%) at 20 years. By 10 years, 58.6% (95% CI: 40.8–72.7%) had progressed to Fontan completion, while 32.5% (95% CI: 18.2–47.6%) had died. In patients achieving Fontan completion, 20-year event-free survival was 73.3% (95% CI: 34.8–91.3%), while 5.0% (95% CI: 0.4–20.5%) had undergone cardiac transplantation and 21.7% (95% CI: 3.2–50.8%) had undergone takedown of the Fontan circulation. Freedom from atrioventricular valve surgery was 57.0% (95% CI: 37.2–72.7%) at 10 and 20 years.

Conclusions:

The association of atrioventricular septal defect and transposition of the great arteries is very rare, and most of these children have unbalanced ventricles. Single-ventricle palliation results in 25-year overall survival of 50%. However, in patients, who had Fontan completion, survival was 75% at 25 years after Fontan operation.

Keywords

Atrioventricular septal defectstransposition of the great arteriessingle-ventricle palliationFontan
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 8 , August 2020 , pp. 1165 - 1170
Copyright
© The Author(s), 2020. Published by Cambridge University Press

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