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Surgical Approaches in Congenital Heart Disease With Congenital Diaphragmatic Hernia: A Multiinstitutional Analysis

Kylie I Holden1, Ashley H Ebanks1, Amir M Khan1

  • 1Departments of Pediatric and Cardiovascular Surgery, McGovern Medical School, University of Texas Health Science Center, Houston, Texas.

The Annals of Thoracic Surgery
|August 23, 2025
PubMed

Insights

Optimal timing for cardiac surgery in infants with congenital heart disease and diaphragmatic hernia is crucial. Performing cardiac repair 30-80 days after congenital diaphragmatic hernia repair improves survival outcomes.

Area of Science:

  • Pediatric Surgery
  • Congenital Anomalies
  • Cardiology

Background:

  • Congenital heart disease (CHD) and congenital diaphragmatic hernia (CDH) often occur together, posing significant surgical challenges.
  • Optimal surgical timing for these combined conditions is not well-defined.

Purpose of the Study:

  • To analyze the relationship between surgical timing and patient outcomes in cases of CDH with concomitant CHD.
  • To provide evidence-based guidance for surgical decision-making in CDH+CHD patients.

Main Methods:

  • Retrospective analysis of 1,886 patients with CDH+CHD from a multi-institutional registry (2007-2022).
  • CDH defect size and cardiac anomalies (stratified by STAT category) were analyzed.
  • Multivariable regression and Loess smoothing models assessed mortality as a function of defect size and surgery timing.

Main Results:

  • Overall mortality was 23.4%. Extracorporeal life support was a significant predictor of in-hospital mortality.
  • Patient mortality correlated with CDH severity (CDHSG stage) within STAT categories.
  • The median time between diaphragmatic repair and cardiac operation was 46 days; survival was greatest when cardiac operations occurred 30-80 days post-CDH repair.

Conclusions:

  • Surgical outcomes for CHD in CDH+CHD patients are influenced by CDH severity and cardiac risk (STAT category).
  • Diaphragmatic repair is typically performed before cardiac repair.
  • Optimal timing for cardiac surgery is 30-80 days after CDH repair, guiding current surgical practices.
Abstract

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