Ann Surg

. 2025 May 20.

 doi: 10.1097/SLA.0000000000006757. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/40391440/

Specific Congenital Heart Disease Diagnoses Impact Congenital Diaphragmatic Hernia Outcomes: The Heart of the Matter

Kylie I Holden ¹, Ashley H Ebanks ¹, Rebecca A Stark ², Yigit S Guner ³, Terry L Buchmiller ⁴, Tim Jancelewicz ⁵, Amir M Khan ⁶, Anthony Johnson ^1 7, Kevin P Lally ¹, Damien J LaPar ⁸, Matthew T Harting ¹; CDH Study Group

Affiliations Expand

• PMID: 40391440
• DOI: 10.1097/SLA.0000000000006757

Abstract

Objective: Our objective was to assess outcomes for newborns with congenital diaphragmatic hernia (CDH) and congenital heart disease (CHD).

Summary background data: CDH is a complex malformation often associated with CHD, yet limited data exist on outcomes for infants with both conditions. This study highlights factors associated with survival and how specific cardiac diagnoses shape patient outcomes, with the goal of improving management strategies.

Methods: This retrospective cohort study used the CDH Study Group (CDHSG) registry to analyze patients with CDH+CHD born from 2007-2022. CDHSG stage, cardiac diagnoses, critical care management, and operative details were assessed. The primary outcome was in-hospital survival, with secondary outcomes related to surgical interventions and extracorporeal life support (ECLS).

Results: 9,257 CDH patients were identified, with 2,013 (21.7%) having CDH+CHD. Excluding isolated arch and unique anomalies, 483 patients had major CHD and 1,131 had minor CHD. Survival for CDH+CHD patients was lower (61.6%) compared to CDH-CHD patients (77.1%) (P<0.001), with longer hospital stays (55 vs. 38 days, P<0.001). Survival was negatively associated with low birthweight (<2.5 kg) (OR=0.40, P<0.0001), larger defect size (OR=0.46, P<0.0001), use of ECLS (OR=0.32, P<0.0001), and prolonged intubation (OR 0.56, P=0.002). Outcomes varied by cardiac diagnosis and CDHSG stage. For single ventricle anatomy, overall survival is 29.0%, but outcomes improve with CDHSG stage. Overall survival for all CDH+CHD by CDHSG stage is: A (97.3%), B (88.4%), C (75.4%), D (52.9%).

Conclusion: CDH+CHD outcomes are associated with CHD diagnosis and CDHSG stage. This study highlights the need for an individualized, risk-stratified approach to managing infants with CDH and CHD, moving from a generalized to a more nuanced, lesion-dependent strategy.

Keywords: CDH study group; Congenital diaphragmatic hernia; congenital heart defects.

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