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

Catherine M Avitabile; Yan Wang; Devon Ash; Sabrina J Flohr; Leny Mathew; Natalie Rintoul; Holly L Hedrick

doi:10.1016/j.jpedsurg.2024.162002

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

J Pediatr Surg. 2024 Oct 12;60(1):162002. doi: 10.1016/j.jpedsurg.2024.162002. Online ahead of print.

Authors

Catherine M Avitabile¹, Yan Wang², Devon Ash², Sabrina J Flohr³, Leny Mathew³, Natalie Rintoul⁴, Holly L Hedrick⁵

Affiliations

¹ Department 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. Electronic address: avitabilec@chop.edu.
² Division of Cardiology and Echocardiography Laboratory Research Unit, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
³ Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
⁴ Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA; Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA; Division of Neonatology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
⁵ Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA; Department of Pediatric General Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

PMID: 39442329
DOI: 10.1016/j.jpedsurg.2024.162002

Abstract

Background: Quantitative echocardiographic (echo) measures of ventricular function predict mortality in pediatric pulmonary hypertension (PH), but studies in congenital diaphragmatic hernia (CDH)-related PH are limited. Few studies report quantitative echo data beyond the first week of life in CDH non-survivors.

Methods: A single-center retrospective, cross-sectional, cohort study included CDH patients born between January 2013 and April 2022 who survived to surgical repair but died during the neonatal hospitalization. Quantitative measures of right (RV) and left ventricular (LV) size and function including tricuspid annular plane systolic excursion Z-score (TAPSEZ), RV fractional area change (FAC), RV/LV ratio, LV eccentricity index, LV M-mode dimensions, and RV/LV systolic strain were performed offline on the last echocardiogram before death. Data were compared between patients who died ≤30 days after repair ("early") vs. >30 days after repair ("late") using the Wilcoxon rank sum test.

Results: Twenty-five (11 early, 14 late) deceased patients had echo images available for analysis. LV size by end-diastolic dimension Z-score was smaller in patients who died early vs. late after repair [-3.03 (-3.93, -2.51) vs. -0.24 (-2.11, 0.53), p = 0.021]. There were trends toward worse RV function (TAPSEZ, RVFAC, RV global and free wall strain) and LV function (apical 4 chamber strain) in patients who died early vs. late after repair.

Conclusion: These preliminary findings support future study of the impact of ventricular hypoplasia and dysfunction on mortality and opportunities for risk stratification based on quantitative echo findings in CDH.

Level of evidence: Cohort study, 4.

Keywords: Congenital diaphragmatic hernia; Echocardiogram; Pulmonary hypertension; Strain.