Pediatr Pulmonol

. 2023 Feb 7.

 doi: 10.1002/ppul.26348. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/36751101/

Long-term exercise and pulmonary function outcomes in a contemporary cohort of children with congenital diaphragmatic hernia

Kimberley G Miles ¹, Adam W Powell ^1 2, Paul J Critser ^1 2, William Hardie ^1 3, Meredith O’Neil ⁴, Michelle Cash ¹, Melissa Magness ¹, Elizabeth Geers ¹, Wayne Mays ¹, Russel Hirsch ^1 2

Affiliations expand

• PMID: 36751101
• DOI: 10.1002/ppul.26348

Abstract

Objective: Congenital diaphragmatic hernia (CDH) survivors are at risk for long-term exercise impairment and pulmonary morbidity, but the generalizability of prior reported cohorts are limited by reduced disease severity and older surgical eras. We assessed the mid-childhood exercise and pulmonary function outcomes in a contemporary cohort of CDH survivors.

Study design: In this retrospective cohort study, we identified 36 consecutive pediatric CDH survivors who underwent cardiopulmonary exercise testing (CPET) and spirometry from 2014 to 2021. Inferential statistics compared survivors with age-, sex-, and size-matched healthy controls; univariate analyses identified factors associated with abnormal testing.

Results: Maximal effort CPET and resting spirometry were completed by 27/36 (75%) and 31/36 (86%) subjects, respectively (median age: 8.1 years [interquartile range: 7.5, 10]; 16 females [44%]). Abnormal CPET (percent predicted [pp] peak VO₂ < 80%) was more common in the CDH cohort (12 vs. 1, p < 0.001) and associated with longer neonatal intensive care unit (NICU) stay (p = 0.02) and oxygen therapy at discharge (p = 0.03). Exercise impairment was mild (pp peak VO₂ 70%-80%), moderate (60%-70%), and severe (<60%) in 6, 4, and 2 survivors, respectively. Abnormal spirometry was more common in the CDH cohort (21 vs. 3, p < 0.001; obstructive [n = 15], restrictive [n = 6]) and associated with decreased gestational age (p = 0.046), longer mechanical ventilation in the NICU (p = 0.02), and orthopedic abnormalities (p = 0.03).

Conclusion(s): Exercise capacity was normal or mildly impaired in most CDH survivors, however, approximately two-thirds demonstrated abnormal spirometry. Impaired exercise capacity and lung function were associated with severity of lung disease postnatally. These data support cardiopulmonary follow-up throughout childhood.

Keywords: cardiopulmonary exercise testing; congenital diaphragmatic hernia; outcomes; spirometry.

© 2023 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.