Ventricular Dysfunction is a Critical Determinant of Mortality in Congenital Diaphragmatic Hernia.

Abstract

RATIONALE Congenital diaphragmatic hernia (CDH) is an anomaly with a high morbidity and mortality. Cardiac dysfunction may be an important and under-recognized contributor to CDH pathophysiology and determinant of disease severity. OBJECTIVES To investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH. METHODS Multicenter, prospectively-collected data in the Congenital Diaphragmatic Hernia Study Group (CDHSG) registry, abstracted between 2015 and 2018, were evaluated. Ventricular function on early echocardiograms, obtained within the first 48 hours of life, was categorized into four hierarchical groups: normal function, right ventricular dysfunction only (RVdys), left ventricular dysfunction only (LVdys), and combined RV and LV dysfunction (RV&LVdys). Univariate, multivariate, and Cox proportional hazards regression analyses were performed. MEASUREMENT AND MAIN RESULTS Cardiac function data from early echocardiograms were available for 1173 (71%) cases and categorized as normal in 711 (61%), RVdys in 182 (15%), LVdys in 61 (5%), and combined RV&LVdys in 219 (19%) cases. Ventricular dysfunction was significantly associated with prenatal diagnosis, CDHSG stage, intrathoracic liver, and patch repair (all p<0.001). Survival varied by category: normal function 80%, RVdys 74%, LVdys 57%, RV&LVdys 51%, p<0.001. The adjusted risk of death (hazard ratio) for cases with LVdys was 1.96 (95% CI 1.29 - 2.98, p=0.020) and for cases with RV&LVdys was 2.27 (95% CI 1.77 - 2.92, p=0.011). All cardiac dysfunction categories were associated with use of extra-corporeal membrane oxygenation (ECMO) (p<0.005). CONCLUSIONS Early ventricular dysfunction occurs frequently in CDH and is an independent determinant of severity and clinical outcome.