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Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia
Robin B Wigen1, Wenming Duan2, Theo J Moraes2
1Division of Pediatric General and Thoracic Surgery, Department of Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Congenital diaphragmatic hernia (CDH) survivors with patch repair and liver up experienced significantly lower lung function. Long-term pulmonary morbidity in CDH patients necessitates ongoing monitoring into adulthood.
Area of Science:
- Pediatric Surgery
- Pulmonology
- Medical Research
Background:
- Congenital diaphragmatic hernia (CDH) is a complex birth defect impacting lung development.
- Long-term outcomes, particularly pulmonary morbidity, require further investigation for effective management.
- Identifying prognostic markers is crucial for predicting and mitigating long-term respiratory issues in CDH survivors.
Purpose of the Study:
- To identify prognostic markers for long-term pulmonary morbidity in congenital diaphragmatic hernia (CDH) survivors.
- To evaluate the impact of surgical repair methods and intraoperative findings on pulmonary function.
- To establish correlations between specific patient characteristics and the degree of pulmonary impairment.
Main Methods:
- Retrospective review of congenital diaphragmatic hernia (CDH) patients treated between 2000 and 2012.
- Recorded data included liver position, use of patch repair, and pulmonary function tests (PFTs).
- Pulmonary function was assessed using forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC), analyzed via ANOVA.
Main Results:
- Patients with patch repair and upward liver position showed significantly lower FEV1 compared to those without patch and downward liver position.
- Lower FEV1 was also observed in patients with patch repair and downward liver position versus those without patch and downward liver position.
- Patients with upward liver and patch repair exhibited moderate lung function reduction, while others had mild to no reduction; obstructive phenotype was noted in older patients.
Conclusions:
- Decreased pulmonary function in congenital diaphragmatic hernia (CDH) survivors is associated with patch repair and liver position.
- These factors serve as potential prognostic markers for long-term respiratory outcomes.
- Continuous monitoring of pulmonary health in adult CDH survivors is recommended.
Purpose:
The aim is to identify prognostic markers of long-term pulmonary morbidity among congenital diaphragmatic hernia (CDH) survivors.
Methods:
A single-institution, retrospective review was performed on all CDH patients from 2000 and 2012 (REB#1000053383). Liver position, patch use, and pulmonary function tests (PFTs) (forced expiratory volume at 1 second [FEV1] and forced vital capacity [FVC] expressed as mean % predicted + SD) were recorded. Data were analyzed using analysis of variance.
Results:
Patients with acceptable and reproducible PFT (n = 72 for 202 total PFT) with patch repair and liver up (n = 28) had significantly lower FEV1 (72.4 + 17.6) than those with no patch and liver down (n = 98, FEV1= 86.3 + 15.9, p = 0.002). Patients with patch repair and liver down (n = 40) also had significantly lower FEV1 (76.6 + 14.4) than those with liver down and no patch (p = 0.0463). Patients with liver up and patch repair had PFT results consistent with moderate reduction of lung function, while the remainder had mild to no decrease in lung function. All CDH patients older than 14 years had a reduction in FEV1/FVC consistent with obstructive phenotype, with a mean FEV1/FVC = 62.3 for patch repair group and FEV1/FVC = 76.1 in the no patch group.
Conclusion:
Decreased pulmonary function of CDH survivors correlated with the use of patch repair and liver position. CDH lung disease should be monitored in adulthood.
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