J Pediatr. 2019 Nov 5. pii: S0022-3476(19)31308-3. doi: 10.1016/j.jpeds.2019.09.072. [Epub ahead of print]

https://www.ncbi.nlm.nih.gov/pubmed/31704056

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

Dao DT¹, Hayden LP², Buchmiller TL³, Kharasch VS⁴, Kamran A³, Smithers CJ⁵, Rice-Townsend SE³, Zalieckas JM³, Becker R⁶, Morash D³, Studley M³, Wilson JM⁷, Sheils CA⁸.

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Abstract

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 (FEV₁pp), percent predicted forced vital capacity (FVCpp), and FEV₁/FVC over time.

RESULTS:

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).

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.

Copyright © 2019 Elsevier Inc. All rights reserved.

KEYWORDS:

FEV(1); FVC; congenital diaphragmatic hernia; linear mixed effects model; pulmonary function testPMID: 31704056 DOI: 10.1016/j.jpeds.2019.09.072