Objective This study aimed to analyze the intraindividual development of lung perfusion in children with congenital diaphragmatic hernia (CDH) at the ages of 2 and 10 years, as well as to investigate prenatal and postnatal influencing factors. Materials and methods Fifty-nine children after CDH were examined as part of a monocentric follow-up program using dynamic contrast-enhanced MRI (DCE-MRI) at 2 years (hereafter referred to as Examination 1, E1) and again at 10 years of age (Examination 2, E2). Pulmonary blood flow (PBF) and pulmonary blood volume (PBV) were quantified separately for each lung. Additionally, prenatal parameters (observed-to-expected fetal lung volume, o/e FLV) and postnatal factors (extracorporeal membrane oxygenation (ECMO); fetoscopic tracheal occlusion (FETO); patch repair; recurrence; and reoperation for recurrence) were recorded. Results Ipsilateral perfusion remained consistently reduced between E1 and E2 (63.4 ± 27.8 vs 62.0 ± 23.6 mL/100 mL/min; p = 0.8001), while PBV significantly decreased (p = 0.0213). Low prenatal o/e FLV values correlated with reduced ipsilateral PBF (E1: r = 0.51; p = 0.0082; E2: r = 0.03; p = 0.0342). Patients who underwent ECMO showed a decrease in contralateral PBF over time (p = 0.0435), and those with FETO tended to exhibit poorer perfusion courses. Conclusion Patients with prenatally more severe lung hypoplasia, particularly those with low o/e FLV, exhibit persistently reduced lung perfusion even in the long term. These ongoing impairments remain stable over time, indicating permanently compromised lung development. Early identification and detailed risk assessment are therefore essential to initiate targeted therapeutic interventions. Key Points <Emphasis Type="BoldItalic">Question</Emphasis> Lung perfusion development in children with CDH between ages 2 and 10, including prenatal and postnatal influencing factors. <Emphasis Type="BoldItalic">Findings</Emphasis> Ipsilateral lung perfusion remained reduced, PBV decreased, and low prenatal o/e FLV correlated with persistently impaired perfusion. <Emphasis Type="BoldItalic">Clinical relevance</Emphasis> Children with severe prenatal lung hypoplasia show lasting perfusion deficits into adolescence. Early risk assessment enables timely, targeted interventions to mitigate long-term pulmonary impairment. Graphical Abstract

Intraindividual development of MR lung perfusion parameters in children after congenital diaphragmatic hernia at 2 and 10 years

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2026年3月15日

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Greta Thater,
Angelika Enns-Ercan,
Julia Elrod,
Schanas Jawhar,
Michael Boettcher,
Thomas Schaible,
Christel Weiß,
Stefan O. Schoenberg,
Meike Weis

摘要

Objective

This study aimed to analyze the intraindividual development of lung perfusion in children with congenital diaphragmatic hernia (CDH) at the ages of 2 and 10 years, as well as to investigate prenatal and postnatal influencing factors.

Materials and methods

Fifty-nine children after CDH were examined as part of a monocentric follow-up program using dynamic contrast-enhanced MRI (DCE-MRI) at 2 years (hereafter referred to as Examination 1, E1) and again at 10 years of age (Examination 2, E2). Pulmonary blood flow (PBF) and pulmonary blood volume (PBV) were quantified separately for each lung. Additionally, prenatal parameters (observed-to-expected fetal lung volume, o/e FLV) and postnatal factors (extracorporeal membrane oxygenation (ECMO); fetoscopic tracheal occlusion (FETO); patch repair; recurrence; and reoperation for recurrence) were recorded.

Results

Ipsilateral perfusion remained consistently reduced between E1 and E2 (63.4 ± 27.8 vs 62.0 ± 23.6 mL/100 mL/min; p = 0.8001), while PBV significantly decreased (p = 0.0213). Low prenatal o/e FLV values correlated with reduced ipsilateral PBF (E1: r = 0.51; p = 0.0082; E2: r = 0.03; p = 0.0342). Patients who underwent ECMO showed a decrease in contralateral PBF over time (p = 0.0435), and those with FETO tended to exhibit poorer perfusion courses.

Conclusion

Patients with prenatally more severe lung hypoplasia, particularly those with low o/e FLV, exhibit persistently reduced lung perfusion even in the long term. These ongoing impairments remain stable over time, indicating permanently compromised lung development. Early identification and detailed risk assessment are therefore essential to initiate targeted therapeutic interventions.

Key Points

Question Lung perfusion development in children with CDH between ages 2 and 10, including prenatal and postnatal influencing factors.

Findings Ipsilateral lung perfusion remained reduced, PBV decreased, and low prenatal o/e FLV correlated with persistently impaired perfusion.

Clinical relevance Children with severe prenatal lung hypoplasia show lasting perfusion deficits into adolescence. Early risk assessment enables timely, targeted interventions to mitigate long-term pulmonary impairment.

Graphical Abstract