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

Research output: Contribution to journalArticle

Authors

External Institution(s)

  • Miller Children's and Women's Hospital
  • Long Beach Memorial Medical Center
  • Emory University
  • University of Minnesota Twin Cities
  • Children’s Healthcare of Atlanta

Details

Original languageEnglish (US)
Pages (from-to)854-863
Number of pages10
JournalCongenital Heart Disease
Volume14
Issue number5
StatusPublished - Sep 1 2019
Peer-reviewedYes

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.

    Research areas

  • Down syndrome, congenital, heart defects, retrospective studies

Citation formats

APA

Peterson, J. K., Setty, S. P., Knight, J. H., Thomas, A. S., Moller, J. H., & Kochilas, L. K. (2019). Postoperative and long-term outcomes in children with Trisomy 21 and single ventricle palliation. Congenital Heart Disease, 14(5), 854-863. https://doi.org/10.1111/chd.12823

Harvard

Peterson, JK, Setty, SP, Knight, JH, Thomas, AS, Moller, JH & Kochilas, LK 2019, 'Postoperative and long-term outcomes in children with Trisomy 21 and single ventricle palliation', Congenital Heart Disease, vol. 14, no. 5, pp. 854-863. https://doi.org/10.1111/chd.12823