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
Johns Hopkins University School of Medicine. University of Texas McGovern Medical School and Children’s Memorial Hermann Hospital.
United States
Journal of Pediatrics
J Pediatr 2023; 259
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.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Genetic Factors Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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