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Published on: February 2, 2021
Intraindividual development of MR lung perfusion parameters in children after congenital diaphragmatic hernia at 2
Greta Thater1, Angelika Enns-Ercan2, Julia Elrod3
1Department of Radiology and Nuclear Medicine, University Medical Center Mannheim, Heidelberg University, Mannheim, Germany. greta.thater@gmx.net.
Insights
Children with congenital diaphragmatic hernia (CDH) show persistently reduced lung perfusion from age 2 to 10. Lower prenatal lung volumes correlate with long-term perfusion deficits, highlighting the need for early risk assessment and intervention.
Area of Science:
- Pediatric Pulmonology
- Medical Imaging
- Congenital Abnormalities
Background:
- Congenital diaphragmatic hernia (CDH) is associated with significant pulmonary hypoplasia and altered lung development.
- Long-term outcomes in CDH survivors often involve respiratory complications, underscoring the need for detailed lung assessment.
Purpose of the Study:
- To analyze the intraindividual development of lung perfusion in children with CDH between ages 2 and 10 years.
- To investigate the influence of prenatal and postnatal factors on lung perfusion in CDH patients.
Main Methods:
- Dynamic contrast-enhanced MRI (DCE-MRI) was used to assess pulmonary blood flow (PBF) and pulmonary blood volume (PBV) in 59 CDH children at ages 2 and 10.
- Prenatal parameters (observed-to-expected fetal lung volume, o/e FLV) and postnatal factors (ECMO, FETO, surgical interventions) were recorded.
Main Results:
- Ipsilateral lung perfusion remained consistently reduced from age 2 to 10 (p=0.8001), while pulmonary blood volume significantly decreased (p=0.0213).
- Low prenatal o/e FLV correlated with reduced ipsilateral PBF at both time points (p<0.05).
- Extracorporeal membrane oxygenation (ECMO) was associated with a decrease in contralateral PBF over time (p=0.0435).
Conclusions:
- Children with CDH and severe prenatal lung hypoplasia exhibit persistently reduced lung perfusion into adolescence.
- These perfusion deficits are stable over time, indicating permanently compromised lung development.
- Early identification of severe lung hypoplasia and comprehensive risk assessment are crucial for timely therapeutic interventions.
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.
