Longitudinal changes of left ventricular hypoplasia and ventricular disproportion in congenital diaphragmatic hernia neonates

Flaminia Pugnaloni, Bartolomeo Bo, Judith Leyens, Irma Capolupo, Andrea Dotta, Pietro Bagolan, Andreas Mueller, Neil Patel, Florian Kipfmueller
Children’s Hospital, University Hospital Bonn and University of Bonn. Bambino Gesù Children’s Hospital-Research Institute, IRCCS. University Medical Center, Mannheim and Heidelberg University. University of Rome “Tor Vergata”. Royal Hospital for Children.
Germany, Italy and United Kingdom

Journal of Perinatal Medicine
J Perinat Med 2025;
DOI: 10.1515/jpm-2025-0327

Abstract
Objectives: Ventricular disproportion, defined as a ratio of right ventricle (RV) end-diastolic diameter to left ventricle (LV) end-diastolic diameter (RVD/LVD) ≥ 1.1 is commonly observed in neonates with congenital diaphragmatic hernia (CDH) and it is independently associated with adverse outcome. Longitudinal postnatal data on ventricular disproportion of CDH neonates are poorly studied and we aimed to evaluate changes in RVD/LVD through serial echocardiographic studies at selected timepoints in the neonatal period.
Methods: This retrospective observational study included CDH neonates admitted to the University Children’s Hospital of Bonn between January 2011 and March 2021. RVD/LVD was measured via apical 4-chamber echocardiographic views at admission, 48 h of life, pre-surgical repair, pre-extubation, and on day 5 of ECMO support, if applicable. Patients receiving palliative care, experiencing early death, or lacking follow-up echocardiographic data were excluded.
Results: Of 248 CDH neonates, 80 were excluded, leaving 168 in the final cohort. At baseline, 41.7 % had an RVD/LVD ≥1.1. Mortality (34.3 %) and ECMO rates (62.9 %) were significantly higher in these patients compared to those with RVD/LVD <1.1. Ventricular disproportion decreased over time: 41.7 % at admission, 23.1 % at 48 h, 15.7 % pre-repair, and 9.1 % pre-extubation. For ECMO patients, RVD/LVD ≥1.1 was found in 62.9 % at admission, decreasing over time. Non-survivors had significantly higher RVD/LVD at 48 h (p=0.020) and pre-extubation (p=0.001).
Conclusions: In CDH neonates, ventricular disproportion improves over time, but RVD/LVD≥1.1 remains strongly associated with mortality, particularly in ECMO patients, where non-survivors exhibit persistently elevated RVD/LVD.

Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Heart Dysfunction Associated with Pulmonary Vascular Disease (Right and Left)
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing

Age Focus: Pediatric Pulmonary Vascular Disease

Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes

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