Abstract
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.
Keywords
congenital diaphragmatic hernia - morbidity - pulmonary function - chronic lung disease
- obstructive lung disease