Postoperative and long-term outcomes in children with Trisomy 21 and single ventricle palliation

• Type of Material: Article
• Authors: Jennifer k. Peterson, MemorialCare Miller Childrens & Womens Hospital; Shaun P. Setty, MemorialCare Miller Childrens & Womens Hospital; Jessica H. Knight, University of Georgia; Amanda S. Thomas, Emory University; James H. Moller, Univ Minnesota; Lazaros Kochilas, Emory University
• Language: English
• Date: 2019-07-22
• Publisher: Wiley
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2019 John Wiley & Sons, Inc. All rights reserved.
• Final Published Version (URL): http://dx.doi.org/10.1111/chd.12823
• Title of Journal or Parent Work: Congenital Heart Disease
• Volume: 14
• Issue: 5
• Start Page: 854
• End Page: 863
• Grant/Funding Information: This work was supported by the National Heart, Lung, and Blood Institute RO1-HL122392 (L.K.K.) and by the Larry and Helen Hoag Foundation (S.P.S., J.K.P.).
• Keywords: heart defects; Pediatric cardiac surgery; Quality; Congenital heart disease; congenital; Mortality; Down syndrome; retrospective studies; Science & Technology; Obstructive sleep apnea; Cardiac & Cardiovascular Systems; Death; Cardiovascular System & Cardiology; Fontan; Repair; Survival; Life Sciences & Biomedicine
• Research Categories: Health Sciences, Epidemiology; Health Sciences, Health Care Management; Biology, Biostatistics

Abstract

Objective: 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. Design: 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 anomaly 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. Results: We identified 118 children with T21 who underwent initial surgical SV palliation. 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 survival 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). Conclusions: 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 select group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long-term survival.