Schanas Jawhar, Greta Thater, Stefan Appelhaus, Michael Boettcher, Julia Elrod, Frank G. Zoellner, Florian Kipfmueller, Marcia Matos Karaorman, Stefan O. Schoenberg, Meike Weis
University Medical Center Mannheim, University Children’s Hospital Mannheim and Heidelberg University.
Germany
European Radiology
Eur Radiol 2026;
DOI: 10.1007/s00330-026-12841-9
Abstract
Objectives: Congenital diaphragmatic hernia (CDH) is a diaphragmatic defect causing lung hypoplasia and frequently resulting in pulmonary arterial hypertension (PAH), associated with high morbidity and mortality. This retrospective study aimed to assess differences in cardiac morphology between ECMO and non-ECMO patients after CDH repair and to evaluate their temporal association with the development of PAH at two and ten years of age.
Materials and methods: In this retrospective study, MRI data from 57 children after CDH repair were analyzed. Patients were divided into two groups: children receiving ECMO therapy (n = 31) and children without ECMO therapy (n = 26). Morphological parameters, including the pulmonary artery to aorta ratio (PA/Ao), right to left ventricle ratio (RV/LV), and the eccentricity index (EI), were measured at two and ten years. Group differences were assessed using independent-samples t-tests, and binary logistic regression was applied. Boxplots visualized parameter distributions.
Results: ECMO-treated patients showed significantly higher EI and RV/LV ratios at two years. The PA/Ao ratio showed no significant difference between groups at this time point. At ten years of age, none of the measured cardiac parameters differed significantly between ECMO and non-ECMO patients. Logistic regression identified ECMO therapy as an independent predictor of elevated EI at two years (OR = 7.68, 95% CI: 1.93-30.47, p = 0.004).
Conclusion: These findings suggest that ECMO-treated children after CDH repair exhibit early signs of persistent pulmonary hypertension, as reflected by elevated EI values at two years. The normalization of cardiac parameters by ten years may indicate pulmonary vascular adaptation over time.
Key points: Question Identifying long-term cardiac alterations after CDH repair is essential for understanding the persistence of pulmonary hypertension, particularly in patients requiring ECMO therapy. Findings Cardiac MRI revealed higher EI and RV/LV ratio in ECMO-treated children at two years, indicating persistent pulmonary hypertension, which normalized by ten years. Clinical relevance Early detection of cardiac remodeling in ECMO-treated CDH survivors enables closer cardiovascular follow-up and may guide timely interventions to support pulmonary vascular adaptation and long-term cardiac health.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
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