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Outcomes of heart surgery in neonates with trisomy 13 and 18: a systematic review with metanalysis

Cecilia de Sá Bittencourt Câmara Bastos1, Ludmilla Vale da Cruz1, Lucas Hirano Arruda Moraes2

  • 1Department of Pediatrics, Faculty of Medicine of the University of São Paulo, Instituto da Criança e do Adolescente do Hospital das Clínicas, Av. Dr. Enéas de Carvalho Aguiar, 647, São Paulo, São Paulo, 05403-000, Brazil.

European Journal of Pediatrics
|June 19, 2025
PubMed

Insights

Cardiac surgery in neonates with trisomy 13 or 18 significantly improves survival and discharge rates compared to palliative care. However, evidence quality is low, necessitating further research and family-centered decision-making.

Area of Science:

  • Medical research
  • Pediatric cardiology
  • Genetics

Background:

  • Trisomy 13 (T13) and Trisomy 18 (T18) are severe genetic disorders often associated with complex congenital heart defects.
  • Management decisions for neonates with T13/T18 and cardiac anomalies involve balancing potential surgical benefits against significant risks and quality of life considerations.

Purpose of the Study:

  • To systematically review and meta-analyze the clinical outcomes of cardiac surgery versus palliative care in neonates with T13 or T18.
  • To assess the impact of surgical intervention on mortality, survival, length of stay, and discharge rates.

Main Methods:

  • A systematic literature search was performed in PubMed and EMBASE databases adhering to PRISMA guidelines.
  • Five retrospective cohort studies involving 1627 neonates with T13 or T18 were included in the meta-analysis.
  • Key outcomes analyzed included in-hospital mortality, 12-month survival, length of stay, hospital discharge, and mechanical ventilation requirements.

Main Results:

  • Cardiac surgery was associated with significantly reduced in-hospital mortality (OR 0.12) and increased 12-month survival (OR 19.77) and hospital discharge rates (OR 12.53).
  • Limited data were available regarding quality of life and duration of mechanical ventilation.
  • The overall quality of evidence was assessed as low due to the retrospective nature and moderate risk of bias in the included studies.

Conclusions:

  • Cardiac surgery demonstrates a significant positive impact on survival and discharge rates for neonates with T13/T18.
  • The low quality of current evidence underscores the need for high-quality studies, including randomized controlled trials.
  • Shared decision-making with families, considering diverse perspectives on quality of life, is crucial in managing these complex cases.

Keywords:
Cardiac surgical proceduresNeonatal intensive care unitsPalliative careTreatment outcomeTrisomy 13 syndromeTrisomy 18 syndrome

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