Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia.

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.