Long-Term Pulmonary Function Outcomes in Children with Pulmonary Hypoplasia

To determine if the underlying cause of pulmonary hypoplasia results in different trajectories of lung growth by describing pulmonary function in 8- to 13-year-old children born with congenital diaphragmatic hernia (CDH), early intervention congenital lung malformation (EICLM), and giant omphalocele (GO).

We performed spirometry and plethysmography as well as echocardiograms in 81 children aged 8- to 13-years with CDH, EICLM, and GO. Clinical and demographic data were collected at the study visit and from electronic medical records. Quantitative analyses of right ventricular function were retrospectively performed.

Fifty-two children with CDH, 17 with EICLM, and 12 with GO were included in the study. Most patients (51.9%) had abnormal lung function. Those with CDH and EICLM were more likely to have an obstructive process, while subjects with GO frequently had restrictive disease. Chest wall abnormalities, patch repair, larger diaphragm defect, and intrathoracic liver position were associated with abnormal lung function in patients with CDH. Abnormal lung function tended to be associated with lower right ventricular function, although this was not statistically significant.

Abnormal lung function persists into late childhood in patients with CDH, EICLM, and GO. However, specific patterns of pulmonary function abnormalities occur in each disorder. These findings suggest that lung growth and remodeling likely differ depending on the underlying cause of pulmonary hypoplasia.