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Lung function over the first 3 years of life in children with congenital diaphragmatic hernia

Howard B Panitch1, Daniel J Weiner2, Rui Feng3

  • 1Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Pediatric Pulmonology
|July 22, 2014
PubMed

Insights

Infants with congenital diaphragmatic hernia (CDH) show persistent abnormal lung function for three years. Lung function impairment in CDH is linked to initial lung hypoplasia and mechanical ventilation duration.

Area of Science:

  • Pediatric Pulmonology
  • Neonatal Surgery
  • Respiratory Physiology

Background:

  • Congenital diaphragmatic hernia (CDH) is associated with variable pulmonary hypoplasia at birth.
  • Limited data exists on long-term lung function in infants surviving CDH.

Purpose of the Study:

  • To assess longitudinal lung function in infants with CDH.
  • To correlate lung function with disease severity and neonatal interventions.
  • To understand lung remodeling in CDH infants.

Main Methods:

  • Pulmonary function testing (PFT) using raised volume rapid thoracic compression.
  • Study included 98 infants with CDH aged 11 days to 44 months.
  • Data collected on demographics and PFT outcomes over 1-5 sessions.

Main Results:

  • Forced expiratory flows were reduced; total lung capacity was normal.
  • Elevated residual volume and functional residual capacity observed.
  • Lower lung function correlated with patch closure, ECMO, pulmonary vasodilators, and longer mechanical ventilation.

Conclusions:

  • CDH survivors exhibit abnormal lung function up to three years of age.
  • Impairment correlates with initial pulmonary hypoplasia and ventilation duration.
  • Findings may inform therapies to optimize lung growth in CDH infants.
Abstract

Keywords:
compensatory lung growthinfant pulmonary function testingneonatal lung diseasepulmonary hypoplasia

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