Open Access

ARTICLE

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

by

1 Children’s Heart Institute, MemorialCare Miller Children’s and Women’s Hospital, Long Beach, California
2 Memorial Heart and Vascular Institute, Long Beach Memorial Medical Center, Long Beach, California
3 Department of Epidemiology and Biostatistics, University of Georgia College of Public Health, Athens, Georgia
4 Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia
5 Departments of Pediatrics and Medicine, University of Minnesota, Minneapolis, Minnesota
6 Children’s Healthcare of Atlanta, Atlanta, Georgia

* Corresponding Author: Shaun P. Setty, Children’s Heart Institute, MemorialCare Miller Children’s and Women’s Hospital, 2801 Atlantic Avenue, Long Beach, CA 90806. Email:

Congenital Heart Disease 2019, 14(5), 854-863. https://doi.org/10.1111/chd.12823

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 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.
Results: 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).
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 se‐ lect group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long‐term survival.

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