Assistant Professor Weill Cornell Medicine Ithaca, New York, United States
Background: Infants with congenital diaphragmatic hernia (CDH) face significant morbidity and mortality. While previous cohort studies have reported improved survival, there's a lack of recent population-level data on infant mortality due to CDH (CDH-IMR), its trends, and geographic variations in the United States. Objective: To analyze temporal trends and geographic variations in CDH-IMR (2007 to 2021) and to assess incidence and survival rates (2016 to 2021) in the US. Design/Methods: From 2007-2021, we used CDC-WONDER linked birth/death data to identify CDH-related infant deaths (ICD-10 code: Q79 as the underlying cause of death), analyze annual CDH-IMR trends, and explore associations with sex, gender, gestational age (GA), and U.S. state of birth. For 2016-2021, we extracted CDC-WONDER natality data to calculate CDH incidence rates, regional variation, and mortality statistics. Descriptive statistics were derived, and bivariate analyses were conducted to discern differences in CDH-IMR by gender, race, and GA. CDH-IMR and incidence rates were expressed per 100,000 live births with Poisson-modeled 95% confidence intervals, and trends were assessed through joinpoint regression via annual percent change (APC). Results: Between 2007 and 2021, 59,117,761 live births and 3391 CDH-related infant deaths were recorded. CDH deaths accounted for 0.96% of all infant deaths and occurred in 0.006% of live births. The mean CDH-IMR was 5.7 (95% CI: 5.5-5.9) per 100,000 live births, with a significant downward trend (APC: -1.3, [95% CI: -2.0, -0.5]). Asian infants had a lower risk of mortality (RR 0.66 [95% CI: 0.55, 0.79]) compared to White infants, and higher GA at birth correlated with better survival. Regional analysis revealed a median CDH-IMR (IQR) of 5.9 (4.7, 7.2), with a three-fold variation across U.S. states. From 2016 to 2021, the median (IQR) incidence rate was 16.3 (10.9, 20.5) per 100,000 live births across states, and mortality in 2021 was significantly lower compared to 2016 (RR 0.73 [95% CI: 0.59, 0.88]).
Conclusion(s): In this national U.S. study, we observed a mean CDH-IMR of 5.7 per 100,000 live births, with a decreasing trend. However, significant variation in CDH mortality persists by region, gender, and race. CDH incidence also varies across states. Study limitations include potential misclassification of causes of death, limited multivariate analysis variables, and absence of CDH incidence data from 2007 to 2015. Despite improvements in care of CDH associated with a decreasing trend in mortality, there remain many opportunities for improvement in outcomes across race, gender and by geographic region.