551 - Changes in Left Ventricular Dysfunction and Impact on Mortality in Congenital Diaphragmatic Hernia

Publication Number: 1530.551

Stephanie Wanamaker, Dell Children's Medical Center of Central Texas, Austin, TX, United States; Marita Thompson, University of Texas at Austin Dell Medical School, Austin, TX, United States; Corey Chartan, University of Texas at Austin Dell Medical School, Austin, TX, United States; Molly M. Pont, University of Texas at Austin Dell Medical School, Austin, TX, United States; Tess Suttles, Dell Children's Medical Center of Central Texas, Austin, TX, United States

Background: Early left ventricular dysfunction is associated with increased mortality and increased requirement of extracorporeal membrane oxygenation (ECMO) in neonates with congenital diaphragmatic hernia (CDH). However, cardiac phenotypes in CDH are dynamic, and how changes in left ventricular function affect outcomes are unknown.

Objective: To assess whether CDH patients with early left ventricular dysfunction that resolve prior to surgical repair have comparable outcomes to those with normal left ventricular function.

Design/Methods: Patients from the CDH Study Group registry from 2015 to 2024 with early-life (0-2 days) and preoperative echocardiograms (0-3 days prior to surgery) with left ventricular function information were included. Patients that were not repaired or had unknown survival status were excluded. Mortality was analyzed using Chi-square test.

Results: 5588 patients were included in the registry. After eliminating patients with missing primary exposure or outcome variables, 1893 patients were included in the final cohort. 288 patients died (15.2%) and 602 patients required ECMO (32%). On early-life echocardiogram, 529 patients had left ventricular dysfunction (27.9%). The proportion of left ventricular dysfunction detected increased over time, from 23.1% in 2015 to 34.5% in 2024 (R=0.79). Patients with early left ventricular dysfunction had 24% mortality compared with 11.8% for those with normal early left ventricular function (p < 0.0001). Of those with early left ventricular dysfunction, 134 patients had persistent left ventricular dysfunction on preoperative echocardiogram (25.3%) and 395 patients had resolved left ventricular dysfunction (74.7%). Those with resolved left ventricular dysfunction had improved mortality to 18.2% compared to 41% for those with persistent left ventricular dysfunction (p < 0.0001), but mortality remained significantly higher than those with normal early left ventricular function (p=0.0009).

Conclusion(s): Congenital diaphragmatic hernia patients with early left ventricular dysfunction that resolve prior to surgical repair have significantly improved mortality than those with persistent left ventricular dysfunction, but mortality remains higher than those with normal early left ventricular function.