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 underrecognized contributor to CDH pathophysiology and determinant of disease severity.Objectives: Our aim was to investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH.Methods: Multicenter, prospectively collected data in the CDH Study Group (CDHSG) registry, abstracted between 2015 and 2018, were evaluated. Ventricular function on early echocardiograms, defined as obtained within the first 48 hours of life, was categorized into four hierarchical groups: normal function, right ventricular dysfunction only (RV_dys), left ventricular dysfunction only (LV_dys), and combined RV and LV dysfunction (RV&LV_dys). Univariate, multivariate, and Cox proportional hazards regression analyses were performed.Measurements and Main Results: Cardiac function data from early echocardiograms were available for 1,173 (71%) cases and categorized as normal in 711 (61%), RV_dys in 182 (15%), LV_dys in 61 (5%), and combined RV&LV_dys 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%; RV_dys, 74%; LV_dys, 57%; and RV&LV_dys, 51% (P < 0.001). The adjusted risk of death (hazard ratio) for cases with LV_dys was 1.96 (95% confidence interval [CI], 1.29-2.98; P = 0.020) and for cases with RV&LV_dys was 2.27 (95% CI, 1.77-2.92; P = 0.011). All cardiac dysfunction categories were associated with use of extracorporeal membrane oxygenation (P < 0.005).Conclusions: Early ventricular dysfunction occurs frequently in CDH and is an independent determinant of severity and clinical outcome.

Keywords: cardiac dysfunction; congenital diaphragmatic hernia; heart; pulmonary hypertension; ventricular dysfunction.