Original ArticleAddressing the causes of late mortality in infants with congenital diaphragmatic hernia

IntroductionDespite improvements of neonatal intensive care, mortality among patients born with congenital diaphragmatic hernia (CDH), remains high, and there is a significant late mortality in this cohort.ObjectiveThe aim of this study was to evaluate the causes of death among 251 consecutive CDH patients treated at our institution during the last 26 years period.MethodsRetrospective review of all causes of death between 1990 and 2015 of the CDH cohort prospectively collected in a database.ResultsOf the 251 CDH patients treated in our department since 1990, 49 were not alive by the end of 2015. Thirty-six patients (14%) died before discharge, and 13 (5%) after the first care event. The mean age at death was 262 days (± 653 days, median 34 days). Eighty six % (42 cases) of the fatalities occurred during the first year of life, more than half of the patients died before 1 month of age and only 13% after the age of 1 year (7 patients) (late mortality). The causes of early mortality (before 1 year of age) were mainly cardio-respiratory, whereas GI complications occurred in the late mortality group.ConclusionThe most common cause of death among CDH patients is respiratory insufficiency and associated pulmonary hypertension, and most of the fatalities occur before 1 year of age. Among older patients, gastrointestinal morbidity as cause of death is highly represented.Level of evidence: II.