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Echocardiographic Changes in Infants with Severe Congenital Diaphragmatic Hernia After Fetoscopic Endoluminal

Catherine M Avitabile^1,2, Sabrina Flohr³, Leny Mathew³

¹Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA. avitabilec@chop.edu.

Pediatric Cardiology

|December 13, 2024

Insights

Fetoscopic endoluminal tracheal occlusion (FETO) in congenital diaphragmatic hernia (CDH) patients shows improved right ventricular function and less left ventricular hypoplasia. FETO impacts cardiac size and function, with better RV systolic function post-repair.

Area of Science:

Cardiology
Pediatric Surgery
Neonatology

Background:

Congenital diaphragmatic hernia (CDH) poses significant survival challenges.
Fetoscopic endoluminal tracheal occlusion (FETO) is a promising intervention to promote lung growth in CDH.
The impact of FETO on postnatal cardiac structure and function remains largely uncharacterized.

Purpose of the Study:

To evaluate the effects of FETO on right ventricular (RV) and left ventricular (LV) size and function in CDH patients.
To compare cardiac parameters between FETO-treated and control CDH patients.
To assess changes in cardiac function over time following FETO and CDH repair.

Main Methods:

Retrospective cohort study comparing 10 FETO patients with 22 control CDH patients.
Quantitative echocardiographic measures including TAPSEZ, RVFAC, RVGLS, RVFWS, RV/LV ratio, LVEI, and LV diastolic/systolic Z-scores were analyzed.
Non-parametric tests and linear regression models were used to compare groups and evaluate changes over time.

Main Results:

FETO patients initially presented with smaller RV/LV ratios and lower LVEI, indicating less RV dilation and septal displacement.
LV hypoplasia was less severe in FETO patients at the first echocardiogram.
Post-repair, FETO patients showed significantly improved RV systolic function (RVFAC, RVGLS, RVFWS) compared to controls.

Conclusions:

FETO influences postnatal cardiac morphology and function in CDH patients.
FETO is associated with reduced RV dilation and LV hypoplasia.
Improved RV systolic function is observed in FETO-treated CDH survivors post-repair.

Read full analysis

Abstract:

Fetoscopic endoluminal tracheal occlusion (FETO) induces lung growth and may improve survival in congenital diaphragmatic hernia (CDH) but the effect on post-natal right (RV) and left (LV) ventricular size and cardiac function is unknown. Quantitative measures of heart size and function including tricuspid annular plane systolic excursion Z-score (TAPSEZ), RV fractional area change (RVFAC), RV global longitudinal and free wall strain (RVGLS, RVFWS), RV/LV ratio, LV eccentricity index (LVEI), and LV M-mode diastolic and systolic Z-scores (LVIDDZ, LVIDSZ) were compared between FETO and control patients on first post-natal echocardiogram, prior to and post CDH repair, and on last available echocardiogram using non-parametric Wilcoxon rank-sum test in a single-center, retrospective cohort study. Linear regression models evaluated change over time, adjusting for clustering and interaction of echocardiogram parameters with time. Thirty-two patients (10 FETO, 22 control) met inclusion criteria. At first echocardiogram, FETO patients demonstrated lower RV/LV ratio and LVEI (p = 0.01 for both) indicating less RV dilation and less ventricular septal displacement, respectively. LV hypoplasia was less severe in FETO patients (p = 0.01 for both LVIDDZ and LVIDSZ) initially. After repair, FETO patients demonstrated better RV systolic function compared to control patients by FAC (p < 0.01), RVGLS (p = 0.02), and RVFWS (p = 0.05). Over time, FETO patients demonstrated greater improvements in RV/LV ratio and LVEI but smaller increases in LV dimensions compared to control patients. Improvements in RV function were similar between the groups. FETO patients demonstrate differences in cardiac size and function compared to control patients.

Keywords:

Congenital diaphragmatic hernia Echocardiogram Pulmonary hypertension Strain

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