Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia

To analyze longitudinal trends of pulmonary function testing in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic.

This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in 1 second (FEV₁pp), percent predicted forced vital capacity (FVCpp), and FEV₁/FVC over time.

Of 268 patients with CDH who survived to discharge, 119 had at least 1 pulmonary function test study. The FEV₁pp (P < .001), FVCpp (P = .017), and FEV₁/FVC (P = .001) decreased with age. Compared with defect size A/B, those with defect size C/D had lower FEV₁pp by an average of 11.5% (95% CI, 2.9%-20.1%; P = .010). A history of oxygen use at initial hospital discharge also correlated with decreased FEV₁pp by an average of 8.0% (95% CI, 1.2%-15.0%; P = .023).

In a select cohort of CDH survivors, average pulmonary function declines with age relative to expected population normative values. Those with severe CDH represent a population at risk for worsening pulmonary function test measurements who may benefit from recognition and monitoring for complications.