Longitudinal Analysis of Pulmonary Function in Survivors of Congenital Diaphragmatic Hernia.

OBJECTIVE

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

STUDY DESIGN

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 (FEVpp), percent predicted forced vital capacity (FVCpp), and FEV/FVC over time.

RESULTS

Of 268 patients with CDH who survived to discharge, 119 had at least 1 pulmonary function test study. The FEVpp (P < .001), FVCpp (P = .017), and FEV/FVC (P = .001) decreased with age. Compared with defect size A/B, those with defect size C/D had lower FEVpp 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 FEVpp by an average of 8.0% (95% CI, 1.2%-15.0%; P = .023).

CONCLUSIONS

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.