TY - JOUR
T1 - Long-Term Survival and Causes of Death in Children with Trisomy 21 After Congenital Heart Surgery
AU - Peterson, Jennifer K.
AU - Kochilas, Lazaros K.
AU - Knight, Jessica
AU - McCracken, Courtney
AU - Thomas, Amanda S.
AU - Moller, James H.
AU - Setty, Shaun P.
N1 - Funding Information:
Funded by The Larry and Helen Hoag Foundation, United States (to S.S., J.P.). Additional support was provided from the National Heart, Lung, and Blood Institute, United States (RO1-HL122392) and the Department of Defense, United States (PR180683 [L.K.]). No funding sources had any role in design and conduct of the study, collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. The data reported here have been supplied by UNOS as the contractor for the Scientific Organ Procurement and Transplantation Network (OPTN). The interpretation and reporting of these data are the responsibility of the authors and in no way should be seen as an official policy of or interpretation by the OPTN or the US Government. The authors declare no conflicts of interest.
Funding Information:
Funded by The Larry and Helen Hoag Foundation, United States (to S.S., J.P.). Additional support was provided from the National Heart, Lung, and Blood Institute, United States ( RO1-HL122392 ) and the Department of Defense, United States ( PR180683 [L.K.]). No funding sources had any role in design and conduct of the study, collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. The data reported here have been supplied by UNOS as the contractor for the Scientific Organ Procurement and Transplantation Network (OPTN). The interpretation and reporting of these data are the responsibility of the authors and in no way should be seen as an official policy of or interpretation by the OPTN or the US Government. The authors declare no conflicts of interest.
Publisher Copyright:
© 2020 Elsevier Inc.
PY - 2021/4
Y1 - 2021/4
N2 - Objective: To evaluate long-term transplant-free survival and causes of death in the trisomy 21 (T21) population after surgery for congenital heart disease (CHD) in comparison with patients who are euploidic. Study design: This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, enriched with prospectively collected data from the National Death Index and the Organ Procurement and Transplantation Network for patients with sufficient direct identifiers. Kaplan-Meier survival plots were generated and multivariable Cox proportional hazards models were used to examine risk factors for mortality between patients with T21 and 1:1 matched patients with comparable CHD who are euploidic. Results: A long-term survival analysis was completed for 3376 patients with T21 (75 155 person-years) who met inclusion criteria. The 30-year survival rate for patients with T21 ranged from 92.1% for ventricular septal defect to 65.3% for complex common atrioventricular canal. Of these, 2185 patients with T21 were successfully matched with a patient who was euploidic. After a median follow-up of 22.86 years (IQR, 19.45-27.14 years), 213 deaths occurred in the T21 group (9.7%) compared with 123 (5.6%) in the euploidic comparators. After adjustment for age, sex, era, CHD complexity, and initial palliation, the hazard ratio of CHD-related mortality was 1.34 times higher in patients with T21 (95% CI, 0.92-1.97; P = .127). Conclusions: CHD-related mortality for patients with T21 after cardiac surgical intervention is comparable with euploidic comparators. Children with T21 require lifelong surveillance for co-occurring conditions associated with their chromosomal abnormality.
AB - Objective: To evaluate long-term transplant-free survival and causes of death in the trisomy 21 (T21) population after surgery for congenital heart disease (CHD) in comparison with patients who are euploidic. Study design: This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, enriched with prospectively collected data from the National Death Index and the Organ Procurement and Transplantation Network for patients with sufficient direct identifiers. Kaplan-Meier survival plots were generated and multivariable Cox proportional hazards models were used to examine risk factors for mortality between patients with T21 and 1:1 matched patients with comparable CHD who are euploidic. Results: A long-term survival analysis was completed for 3376 patients with T21 (75 155 person-years) who met inclusion criteria. The 30-year survival rate for patients with T21 ranged from 92.1% for ventricular septal defect to 65.3% for complex common atrioventricular canal. Of these, 2185 patients with T21 were successfully matched with a patient who was euploidic. After a median follow-up of 22.86 years (IQR, 19.45-27.14 years), 213 deaths occurred in the T21 group (9.7%) compared with 123 (5.6%) in the euploidic comparators. After adjustment for age, sex, era, CHD complexity, and initial palliation, the hazard ratio of CHD-related mortality was 1.34 times higher in patients with T21 (95% CI, 0.92-1.97; P = .127). Conclusions: CHD-related mortality for patients with T21 after cardiac surgical intervention is comparable with euploidic comparators. Children with T21 require lifelong surveillance for co-occurring conditions associated with their chromosomal abnormality.
KW - congenital heart surgery
KW - genetics
KW - outcomes
KW - statistics (survival analysis)
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UR - http://www.scopus.com/inward/citedby.url?scp=85099840051&partnerID=8YFLogxK
U2 - 10.1016/j.jpeds.2020.12.058
DO - 10.1016/j.jpeds.2020.12.058
M3 - Article
C2 - 33359302
AN - SCOPUS:85099840051
SN - 0022-3476
VL - 231
SP - 246-253.e3
JO - Journal of Pediatrics
JF - Journal of Pediatrics
ER -