@Article{chd.12823,
AUTHOR = {Jennifer K. Peterson, Shaun P. Setty, Jessica H. Knight, Amanda S. Thomas, James H. Moller, Lazaros K. Kochilas},
TITLE = {Postoperative and long‐term outcomes in children with Trisomy 21 and single ventricle palliation},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {14},
YEAR = {2019},
NUMBER = {5},
PAGES = {854--863},
URL = {http://www.techscience.com/schd/v14n5/38859},
ISSN = {3071-1738},
ABSTRACT = {<b>Objective:</b> Patients with Trisomy 21 (T21) and single ventricle (SV) physiology 
present unique challenges compared to euploidic counterparts. This study reports 
postoperative and long‐term outcomes in patients with T21 and SV palliation.<br/>
<b>Design:</b> This retrospective cohort study from the Pediatric Cardiac Care Consortium 
(PCCC) included patients with T21 (<21 years old) that underwent surgical palliation 
for SV between 1982 and 2008 and control patients without known genetic anom‐
aly following Fontan palliation for similar diagnoses. Kaplan‐Meier survival plots 
were created based on death events obtained from the PCCC and by linkage with 
the National Death Index (NDI) and the Organ Procurement and Transplantation 
Network (OPTN) through 2014 for patients with adequate identifiers.<br/>
<b>Results:</b> We identified 118 children with T21 who underwent initial surgical SV pallia‐
tion. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent 
Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in‐hospital sur‐
vival of 80.3% and 76.0%, respectively. Fifty‐three patients had sufficient identifiers 
for PCCC‐NDI‐OPTN linkage. Ten‐year survival, conditioned on discharge alive after 
the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic 
anomaly (P = .001). Median age at death for T21 patients following initial surgical SV 
palliation was 2.69 years (IQR 1.34‐7.12) with most deaths (89.2%) attributed to the 
underlying congenital heart disease (CHD).<br/>
<b>Conclusions:</b> Children with T21 and SV are at high risk for procedural and long‐term 
mortality related to their genetic condition and underlying CHD. Nevertheless, a se‐
lect group of patients can successfully complete Glenn or Fontan palliation, reaching 
satisfactory long‐term survival.},
DOI = {10.1111/chd.12823}
}