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Ventricular Dysfunction in Patients With Congenital Diaphragmatic Hernia Who Die After Repair

Catherine M Avitabile1, Yan Wang2, Devon Ash2

  • 1Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA; Division of Cardiology and Echocardiography Laboratory Research Unit, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Journal of Pediatric Surgery
|October 23, 2024
PubMed

Insights

Smaller left ventricular size in neonates with congenital diaphragmatic hernia (CDH) predicted early mortality after surgical repair. Echocardiography may help stratify risk in these critical patients.

Area of Science:

  • Pediatric Cardiology
  • Neonatal Critical Care
  • Congenital Abnormalities

Background:

  • Pulmonary hypertension (PH) in pediatric patients with congenital diaphragmatic hernia (CDH) is associated with mortality.
  • Quantitative echocardiography (echo) is crucial for assessing ventricular function in PH, but data in CDH survivors are limited.
  • Few studies report quantitative echo data in CDH non-survivors beyond the first week of life.

Purpose of the Study:

  • To investigate quantitative echocardiographic measures of ventricular size and function in neonatal CDH patients who died during hospitalization.
  • To compare echo findings between early (<30 days) and late (>30 days) deaths after surgical repair in CDH patients.

Main Methods:

  • Retrospective, cross-sectional cohort study of CDH patients (2013-2022) who died during neonatal hospitalization after surgical repair.
  • Quantitative echo measures of right (RV) and left ventricular (LV) size and function were analyzed offline on the last echocardiogram before death.
  • Statistical comparison (Wilcoxon rank sum test) of echo data between early and late death groups.

Main Results:

  • Twenty-five deceased CDH patients (11 early, 14 late deaths) were analyzed.
  • Patients who died early had significantly smaller LV end-diastolic dimension Z-scores compared to late deaths.
  • Trends indicated worse RV and LV function in the early death group, including reduced TAPSEZ, RVFAC, and strain parameters.

Conclusions:

  • Ventricular hypoplasia and dysfunction may impact mortality in CDH patients.
  • Quantitative echocardiography findings show potential for risk stratification in CDH, warranting further investigation.
Abstract

Keywords:
Congenital diaphragmatic herniaEchocardiogramPulmonary hypertensionStrain

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