Associate Professor of Clinical Pediatrics Weill Cornell Medicine Ithaca, New York, United States
Background: Congenital lung malformations (CLM) are developmental anomalies due to cellular signaling dysregulation with varied presentations during newborn period. Prospective registries suggest increasing incidence of CLM in developed countries (Stocker et al, 2015; Lau et al 2017) and data is lacking regarding outcomes including mortality due to symptomatic CLMs. Objective: To assess temporal trends, demographics and interstate variations in the infant mortality rate (IMR) associated with CLM in the United States. Design/Methods: Using the CDC-WONDER birth/infant death linked database from 1999 to 2020, we analyzed temporal trends and interstate differences in infant mortality attributed to CLM, utilizing the ICD-10 code: Q33 –Congenital malformations of lung. IMR data was presented per 100,000 live births, with Poisson-modeled 95% confidence intervals (CI). Trends and annual percentage changes (APCs) were determined using Joinpoint regression 5.1 (National Cancer Institute, USA). We conducted bivariate analysis to examine IMR based on gender, race and gestational age with statistical significance set at p<.05. Results: Between 2007 and 2021, out of 59,117,761 births, 4,763 (0.008%) infants died due to CLM. Majority (81%) of these fatalities occurred in first week of life. Male infants (59%) had higher mortality rates of 9.2 per 100,000 live births (Relative Risk 1.35 [95%CI: 1.27, 1.43], P<.001). Asian infants exhibited higher and Black infants showed lower mortality risk, compared to White infants [Table 1]. The median incidence of CLM-IMR across states was 7.8 (IQR: 6.4, 9.5) per 100,000 live births [Figure 1]. A 2.4 fold variation in CLM related infant mortality incidence was observed among different states in the United States. No statistically significant decline in mortality rates observed during study period (APC: -0.35% [95% CI: -1.2, 0.57], p=0.45).[Figure 2]
Conclusion(s): Over the last 15 years, the incidence of CLM related infant mortality did not change across United States. However, considerable variation in survival exists across the states and by race. Registry based incidence and mortality data, stratified by types of CLM, may offer better insights into long term outcomes and uniform data on pulmonary morbidities are still lacking regarding quality of life in the United States.
Figure 1. Interstate Variation in Infant Mortality Attributable to Congenital Lung Malformations in United States, 2007, 2021