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Pulmonary Morbidity in Congenital Diaphragmatic Hernia Survivors Treated at a Non-ECMO Center From 1998 to 2015: A
Ulla Lei Larsen1,2,3, Lone Agertoft4, Anne Maria Herskind5
1Research Unit for Department of Anaesthesiology & Intensive Care, Odense University Hospital, Odense, Denmark.
Insights
Congenital diaphragmatic hernia (CDH) survivors frequently experience mild obstructive lung impairment and hyperinflation. Long-term follow-up by a multidisciplinary team is recommended for these CDH patients.
Area of Science:
- Pediatric Pulmonology
- Thoracic Surgery
- Medical Imaging
Background:
- Congenital diaphragmatic hernia (CDH) is characterized by lung hypoplasia, pulmonary hypertension, and cardiac dysfunction.
- Pulmonary status in CDH survivors is influenced by multiple factors including hypoplasia severity and ventilator-induced injury.
- The long-term pulmonary sequelae in CDH patients remain incompletely understood.
Purpose of the Study:
- To describe the pulmonary status of congenital diaphragmatic hernia survivors.
- To evaluate risk factors associated with pulmonary outcomes in this population.
Main Methods:
- A cohort of CDH survivors (1998-2015) underwent pulmonary function tests and chest X-rays.
- Lung function tests included spirometry with bronchodilator response, body plethysmography, and diffusion capacity measurements.
- Chest X-rays assessed for complications such as hernia recurrence and scoliosis.
Main Results:
- Of 71 eligible survivors, 51 participated (71.8%) with a median age of 12.2 years.
- Mild obstructive lung impairment (43.8%) and hyperinflation were common findings.
- Increased residual volume to total lung capacity ratio and reduced forced expiratory volume in 1 second were associated with longer mechanical ventilation duration.
Conclusions:
- Mild obstructive impairment and hyperinflation are frequent in the CDH cohort.
- Restrictive lung disorders were identified in a small subset of patients.
- Lifelong follow-up by a specialized multidisciplinary team is advocated for CDH survivors.
Introduction:
A main feature of CDH is lung hypoplasia and the related presentation of pulmonary hypertension and cardiac dysfunction. Multiple factors influence pulmonary status after CDH: degree of hypoplasia, ventilator-induced injury, altered growth and development of pulmonary structures, reduced diaphragm function and chest wall abnormalities. The evolution of pulmonary sequela in this population is still unclear. We aimed to describe the pulmonary status of our population of CDH-survivors and evaluated on risk factors.
Methods:
CDH-survivors (1998-2015) were included and performed lung function tests and chest X-rays.
Results:
Fifty-one (51/71, 71.8%) participated. Median age was 12.2 (5.5-21.4) years, 28 (54.9%) male, 42 (82.4%) had left-sided hernias, 10 (19.6%) needed patch-repair and median length of stay in hospital was 28.0 (IQR 18.5-61.6) days in Table 1. Spirometry including bronchodilator response (BDR)-test, body plethysmography, and diffusion capacity, were available for 48, 42, and 40 participants. The mean (SD) z-score for FEV1 and FVC was -0.26 (1.70) and -0.28 (1.70). Twenty-one (43.8%) had obstructive patterns and six had positive BDR. TLC mean (SD) z-score was -0.18 (1.10). Four showed restricted/mixed patterns and 13 showed signs of hyperinflation. Increased RV/TLC-ratio and reduced FEV1 was associated with longer time on mechanical ventilation. Diffusion capacity was decreased in three cases. Chest X-ray revealed hernia recurrence (13.9%) and scoliosis (38.9%).
Conclusion:
Mild obstructive impairment and hyperinflation was frequent in our CDH cohort and only a small subset of restrictive disorders were identified. We advocate follow-up by a specialized multidisciplinary team through childhood and into adulthood.
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