Sex-Specific Differences in Congenital Diaphragmatic Hernia Mortality

Shelby R Sferra; Matthew Guo; Andres J Gonzalez Salazar; Annalise B Penikis; Abigail J Engwall-Gill; Ashley Ebanks; Matthew T Harting; Joseph M Collaco; Shaun M Kunisaki; CDH Study Group

doi:10.1016/j.jpeds.2023.113481

Sex-Specific Differences in Congenital Diaphragmatic Hernia Mortality

J Pediatr. 2023 Aug:259:113481. doi: 10.1016/j.jpeds.2023.113481. Epub 2023 May 16.

Authors

Shelby R Sferra¹, Matthew Guo¹, Andres J Gonzalez Salazar¹, Annalise B Penikis¹, Abigail J Engwall-Gill¹, Ashley Ebanks², Matthew T Harting², Joseph M Collaco³, Shaun M Kunisaki⁴; CDH Study Group

Affiliations

¹ Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD.
² Department of Pediatric Surgery, University of Texas McGovern Medical School and Children's Memorial Hermann Hospital, Houston, TX.
³ Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, MD.
⁴ Division of General Pediatric Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD. Electronic address: skunisa1@jhmi.edu.

PMID: 37196780
DOI: 10.1016/j.jpeds.2023.113481

Abstract

Objective: To compare disease severity and mortality differences between female and male patients with congenital diaphragmatic hernia (CDH).

Study design: We queried the CDH Study Group (CDHSG) database for CDH neonates managed between 2007 and 2018. Female and males were compared in statistical analyses using t tests, χ² tests, and Cox regression, as appropriate (P ≤ .05).

Results: There were 7288 CDH patients, of which 3048 (41.8%) were female. Females weighed less on average at birth than males (2.84 kg vs 2.97 kg, P < .001) despite comparable gestational age. Females had similar rates of extracorporeal life support (ECLS) utilization (27.8% vs 27.3%, P = .65). Although both cohorts had equivalent defect size and rates of patch repair, female patients had increased rates of intrathoracic liver herniation (49.2% vs 45.9%, P = .01) and pulmonary hypertension (PH) (86.6% vs 81.1%, P < .001). Females had lower survival rates at 30-days (77.3% vs 80.1%, P = .003) and overall lower survival to discharge (70.2% vs 74.2%, P < .001). Subgroup analysis revealed that increased mortality was significant among those who underwent repair but were never supported on ECLS (P = .005). On Cox regression analysis, female sex was independently associated with mortality (adjusted hazard ratio 1.32, P = .02).

Conclusion: After controlling for the established prenatal and postnatal predictors of mortality, female sex remains independently associated with a higher risk of mortality in CDH. Further study into the underlying causes for sex-specific disparities in CDH outcomes is warranted.

Keywords: congenital diaphragmatic hernia; disparities; mortality.

MeSH terms

Female
Hernias, Diaphragmatic, Congenital*
Humans
Hypertension, Pulmonary*
Infant, Newborn
Male
Pregnancy
Proportional Hazards Models
Retrospective Studies
Survival Rate
Treatment Outcome