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Author Notes:

Corresponding author: Shaun P. Setty, MD, Memorial Heart & Vascular Institute, 2801 Atlantic Avenue, Long Beach, CA 90806, ssetty@memorialcare.org , telephone 562-933-3325, fax 562-933-3330


Research Funding:

Helen E. Hoag Pediatric Cardiac Surgery Research Endowment.

Partial support: National Heart, Lung, and Blood Institute grant 5R01 HL122392-04, PI Lazaros K. Kochilas.

The authors acknowledge the support of the staff from the Pediatric Cardiac Care Consortium staff and the participating centers.


  • Science & Technology
  • Life Sciences & Biomedicine
  • Cardiac & Cardiovascular Systems
  • Respiratory System
  • Surgery
  • Cardiovascular System & Cardiology

Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease Interventions


Journal Title:

Annals of Thoracic Surgery


Volume 103, Number 6


, Pages 1941-1949

Type of Work:

Article | Post-print: After Peer Review


Background The purpose of this study is to report short- and long-term outcomes after congenital heart defect (CHD) interventions in patients with trisomy 13 or 18. Methods A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) identified children with trisomy 13 or 18 with interventions for CHD between 1982 and 2008. Long-term survival and cause of death were obtained through linkage with the National Death Index. Results A total of 50 patients with trisomy 13 and 121 patients with trisomy 18 were enrolled in PCCC between 1982 and 2008; among them 29 patients with trisomy 13 and 69 patients with trisomy 18 underwent intervention for CHD. In-hospital mortality rates for patients with trisomy 13 or trisomy 18 were 27.6% and 13%, respectively. Causes of in-hospital death were primarily cardiac (64.7%) or multiple organ system failure (17.6%). National Death Index linkage confirmed 23 deaths after discharge. Median survival (conditioned to hospital discharge) was 14.8 years (95% confidence interval [CI]: 12.3 to 25.6 years) for patients with trisomy 13 and 16.2 years (95% CI: 12 to 20.4 years) for patients with trisomy 18. Causes of late death included cardiac (43.5%), respiratory (26.1%), and pulmonary hypertension (13%). Conclusions In-hospital mortality rate for all surgical risk categories was higher in patients with trisomy 13 or 18 than that reported for the general population. However, patients with trisomy 13 or 18, who were selected as acceptable candidates for cardiac intervention and who survived CHD intervention, demonstrated longer survival than previously reported. These findings can be used to counsel families and make program-level decisions on offering intervention to carefully selected patients.

Copyright information:

© 2017 The Society of Thoracic Surgeons

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