Ventricular Dysfunction May Predict Survival in Congenital Diaphragmatic Hernia

Ventricular dysfunction that occurs in newborns within 48 hours of life with congenital diaphragmatic hernia (CDH) is common and may represent an independent indicator of disease severity and associated survival, study results published in the American Journal of Respiratory and Critical Care Medicine suggest.

Researchers included data from the multicenter Congenital Diaphragmatic Hernia Study Group (CDHSG) registry pulled between 2015 and 2018. A total of 1646 CDH cases were included in the analysis. A total of 4 hierarchical groups were used to categorize ventricular function on early echocardiograms (ie, echocardiograms obtained within the first 48 hours of life). These categories were: normal function, right ventricular (RV) dysfunction, left ventricular (LV) dysfunction, and combined RV and LV dysfunction. The primary outcome of interest was survival to discharge. Secondary outcomes included duration of intubation, length of admission at surgical center for survivors, and use of extracorporeal membrane oxygenation (ECMO).

Approximately 71% (n=1173) of patients had available cardiac function data from early echocardiograms. Normal ventricular function, RV dysfunction, LV dysfunction, and combined RV and LV dysfunction comprised 61%, 15%, 5%, and 19% of cases, respectively. There were significant associations between ventricular dysfunction and prenatal diagnosis (P =.007), CDHSG stage (P <.001), intrathoracic liver (P <.001), and patch repair (P <.001).

In 1172 cases, the median age at discharge of survivors was 42 days (range, 3-719 days). Significant differences were found in survival between the normal function, RV dysfunction, LV dysfunction, and combined RV and LV dysfunction groups (80% vs 74% vs 57% vs 51%, respectively; P <.001).

According to the adjusted analysis, the hazard ratio for death among newborns with LV dysfunction was 1.96 (95% CI, 1.29-2.98; P =.020), whereas the risk for death was 2.27 (95% CI, 1.77-2.92; P =.011) among newborns with both RV and LV dysfunction. Use of ECMO was associated with RV dysfunction (P =.003), LV dysfunction (P <.001), and combined RV and LV dysfunction (P <.001) at early echocardiography.

Study limitations included the observational nature of the data, as well as the somewhat subjective and varied nature of ventricular dysfunction definitions by clinician and/or center.

“Early ventricular function should be routinely evaluated in all cases of CDH,” the researchers wrote, “to improve understanding of individual pathophysiology and inform clinical decision-making.”

Reference

Patel N, Lally PA, Kipfmueller F, et al; for the Congenital Diaphragmatic Hernia Study Group. Ventricular dysfunction is a critical determinant of mortality in congenital diaphragmatic hernia [published online August 13, 2019]. Am J Respir Crit Care Med. doi:10.1164/rccm.201904-0731OC