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Abnormalities in pulmonary function in infants with high-risk congenital diaphragmatic hernia

Michal Rygl1, Petra Rounova1, Jan Sulc2

  • 1Department of Paediatric Surgery, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic.

Biomedical Papers of the Medical Faculty of the University Palacky, Olomouc, Czechoslovakia
|September 15, 2015
PubMed

Insights

Infants surviving congenital diaphragmatic hernia (CDH) often show peripheral airway obstruction and altered lung function. Unfavorable prognostic factors like Gore-Tex patches or pulmonary hypertension worsen these pulmonary issues in CDH survivors.

Area of Science:

  • Pediatric Pulmonology
  • Neonatal Surgery
  • Medical Engineering

Background:

  • Congenital diaphragmatic hernia (CDH) is a severe birth defect impacting lung development.
  • Infant pulmonary function tests (IPFT) are crucial for assessing respiratory health in young CDH survivors.
  • Identifying prognostic factors is key to managing long-term respiratory outcomes in CDH.

Purpose of the Study:

  • To analyze lung growth and infant pulmonary function test (IPFT) abnormalities in congenital diaphragmatic hernia (CDH) survivors under three years old.
  • To investigate the correlation between unfavorable prognostic factors and pulmonary function in CDH survivors.
  • To provide insights into the long-term respiratory sequelae of CDH.

Main Methods:

  • Thirty high-risk CDH survivors (age 1.32±0.54 years) underwent comprehensive IPFT, including tidal breathing analysis, baby plethysmography, and rapid thoraco-abdominal compression.
  • Patients were categorized based on surgical repair (Gore-Tex patch) and presence of pulmonary hypertension (iNO group).
  • Statistical analysis compared IPFT results against reference values and correlated findings with prognostic factors.

Main Results:

  • A high incidence of peripheral airway obstruction (70%) was observed in CDH survivors.
  • Increased functional residual capacity (FRCp) and effective airway resistance (Reff), along with decreased specific compliance (Crs/kg), were significant findings.
  • Gore-Tex patch use and pulmonary hypertension were associated with more severe alterations in FRCp.

Conclusions:

  • CDH survivors exhibit a high prevalence of peripheral airway obstruction, increased FRCp, and reduced respiratory compliance.
  • Unfavorable prognostic factors, specifically Gore-Tex patch repair and pulmonary hypertension, are linked to more pronounced pulmonary function deficits.
  • These findings underscore the need for close monitoring and tailored respiratory support for CDH survivors.
Abstract

Keywords:
chronic lung morbiditycongenital diaphragmatic herniainfant pulmonary function testinglung dysfunction

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