ArticleCongenital heart disease2019
Postoperative and long-term outcomes in children with Trisomy 21 and single ventricle palliation.
Article in Congenital heart disease, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- Derivation and Validation of a General Predictive Model for Long Term Risks for Mortality and Invasive Interventions in Congenital and Acquired Cardiac Conditions Encountered in the Young.Pediatric cardiology · 2023Trial
- Treatment Options Offered to Infants with Single Ventricle Physiology and Genetic Aneuploidy: A Survey of US Pediatric Intensivists, Cardiologists, and Cardiothoracic Surgeons.Pediatric cardiology · 2026Article
- Anaesthesia for the child with trisomy 21.BJA education · 2024Review
- Trisomy 21 and Congenital Heart Disease: Impact on Health and Functional Outcomes From Birth Through Adolescence: A Scientific Statement From the American Heart Association.Journal of the American Heart Association · 2024Review
- Unexpected Growth of Small Right Ventricle After Failed Repair of Atrioventricular Septal Defect.Annals of thoracic surgery short reports · 2024Article
- Reintervention Before Bidirectional Cavopulmonary Shunt and Intermediate Outcomes in Children with Single Ventricle Who Underwent Main Pulmonary Artery Banding.Pediatric cardiology · 2023Article
- Lesion-Specific Congenital Heart Disease Mortality Trends in Children: 1999 to 2017.Pediatrics · 2022Article
- Lesion-specific mortality due to congenital heart disease in U.S. adults from 1999 to 2017.Birth defects research · 2022Article
- Hemodynamic Characteristics After Fontan Procedure in Patients with Down's Syndrome.Pediatric cardiology · 2022Article
- Trisomy 21 Patients Undergoing Cavopulmonary Connections Need Improved Preoperative and Postoperative Care.The Annals of thoracic surgery · 2021Observational
- Long-Term Survival and Causes of Death in Children with Trisomy 21 After Congenital Heart Surgery.The Journal of pediatrics · 2021Article
- Down syndrome and congenital heart disease: perioperative planning and management.Journal of congenital cardiology · 2021Review
Corrections and comments
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Authors and funding
6 authors.
Funding
Abstract
objectivePatients 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.
designThis 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.
resultsWe 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).
conclusionsChildren 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.
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