Early postnatal echocardiographic characteristics impact survival and extracorporeal life support in congenital diaphragmatic hernia.

Abstract

Background: Early echocardiographic characteristics (EC) of congenital diaphragmatic hernia (CDH) neonates and their associations with outcomes, especially differences by laterality and size, are unknown.

Methods: Congenital Diaphragmatic Hernia Study Group data between 2015 and 2020 were used. Early postnatal EC, including atrial and ductal shunt direction, pulmonary hypertension (PH) severity, and ventricular size and function, were assessed based on defect laterality and size. Outcomes included mortality and extracoporeal life support (ECLS) use.

Results: The study population included 1777 infants. Severe PH, right-to-left shunt, left ventricular (LV) hypoplasia, right ventricular dilation, and ventricular dysfunction were more prevalent in larger defects. Independent of defect size, neonates with R-CDH had more severe PH, more bidirectional and right-to-left atrial shunt, and more biventricular (BV) dysfunction. In contrast, L-CDH neonates had more LV hypoplasia and left-to-right atrial shunt. After adjusting for defect side, larger defects were associated with LV hypoplasia and right-to-left and bidirectional atrial shunt. In multivariate analysis, right-to-left atrial shunt and BV dysfunction were associated with increased mortality, whereas bidirectional atrial shunt and BV dysfunction were associated with ECLS use.

Conclusions: CDH neonates are at increased risk for early cardiac dysfunction. EC differ by laterality and size. Management of cardiac dysfunction in CDH may improve outcomes.

Impact: Cardiac dysfunction has emerged as a factor contributing to adverse outcomes in congenital diaphragmatic hernia (CDH). However, there are limited data on the impact of defect size, laterality, and severity of postnatal cardiac dysfunction on outcomes. Echocardiographic characteristics in the first two days of life differ by defect laterality and size. Right-to-left atrial shunt and biventricular dysfunction are associated with increased mortality. Bidirectional atrial shunt and biventricular dysfunction were associated with extracorporeal life support use. Our results support the need for standardized cardiac function assessment in critically ill neonates with CDH. Future strategies to identify and manage these diverse hemodynamic profiles are needed to improve outcomes.