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Updated: Jan 21, 2026

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
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Postoperative and long-term outcomes in children with Trisomy 21 and single ventricle palliation

Jennifer K Peterson1, Shaun P Setty1,2, Jessica H Knight3

  • 1Children's Heart Institute, MemorialCare Miller Children's and Women's Hospital, Long Beach, California.

Congenital Heart Disease
|July 24, 2019
PubMed

Insights

Children with Trisomy 21 (T21) and single ventricle (SV) heart conditions face high mortality risks. However, some T21 patients can achieve satisfactory long-term survival after SV palliation procedures like the Fontan operation.

Area of Science:

  • Pediatric Cardiology
  • Genetics
  • Congenital Heart Disease

Background:

  • Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges in pediatric cardiac surgery.
  • Outcomes for these patients are often compared to euploidic counterparts.

Purpose of the Study:

  • To report postoperative and long-term outcomes in patients with T21 undergoing SV palliation.
  • To compare survival rates of T21 patients with SV to a control group without genetic anomalies.

Main Methods:

  • Retrospective cohort study using the Pediatric Cardiac Care Consortium (PCCC) database.
  • Inclusion of T21 patients (<21 years) undergoing SV palliation (1982-2008) and controls without genetic anomalies post-Fontan.
  • Kaplan-Meier survival analysis using PCCC, National Death Index (NDI), and Organ Procurement and Transplantation Network (OPTN) data.

Main Results:

  • 118 children with T21 underwent initial SV palliation; 90 survived first surgery.
  • In-hospital survival for Glenn and Fontan palliation was 80.3% and 76.0%, respectively.
  • Ten-year survival post-Fontan was 66.7% for T21 patients versus 92.2% for controls (P=.001); median age at death was 2.69 years, primarily due to congenital heart disease.

Conclusions:

  • Children with T21 and SV have high procedural and long-term mortality risks.
  • A subset of these patients can achieve satisfactory long-term survival after successful Glenn or Fontan palliation.
  • The genetic condition and underlying congenital heart disease significantly impact outcomes.
Abstract

Keywords:
Down syndromecongenitalheart defectsretrospective studies

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