Management Practice and Mortality for Infants with Congenital Diaphragmatic Hernia

ObjectiveCongenital diaphragmatic hernia (CDH) is fatal in 20 to 40% of cases, largely due to pulmonary dysmaturity, lung hypoplasia, and persistent pulmonary hypertension. Evidence for survival benefit of inhaled nitric oxide (iNO), extracorporeal membrane oxygenation (ECMO), and other medical interventions targeting pulmonary hypertension is lacking. We assessed medical interventions and mortality over time in a large multicenter cohort of infants with CDH. Study DesignWe identified all infants34 weeks' gestation with CDH discharged from 29 neonatal intensive care units between 1999 and 2012 with an average of2 CDH admissions per year. We examined mortality and the proportion of infants exposed to medical interventions, comparing four periods of time: 1999-2001, 2002-2004, 2005-2007, and 2008-2012. ResultsWe identified 760 infants with CDH. From 1999-2001 to 2008-2012, use of iNO increased from 20% of infants to 50%, sildenafil use increased from 0 to 14%, and milrinone use increased from 0 to 22% (p<0.001). Overall mortality (28%) did not significantly change over time compared with the earliest time period. ConclusionDespite changing use of iNO, sildenafil, and milrinone, CDH mortality has not significantly decreased in this population of infants.