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An association between pulmonary hypertension and impaired lung function in infants with congenital diaphragmatic

Fiona Healy1, Wei Lin2, Rui Feng2

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

Pediatric Pulmonology
|March 14, 2014
PubMed

Insights

Infants with congenital diaphragmatic hernia and pulmonary hypertension show increased lung overinflation. These findings suggest therapies targeting lung development could improve outcomes for infants with CDH and PH.

Area of Science:

  • Pediatric Pulmonology
  • Neonatal Research
  • Congenital Diaphragmatic Hernia Research

Background:

  • Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypertension (PH) due to abnormal pulmonary artery development.
  • Pulmonary vascular growth in CDH patients shows significant morphological variation, impacting lung development.

Purpose of the Study:

  • To determine if infants with CDH and PH exhibit more significant abnormalities in infant pulmonary function testing (IPFT) compared to those without PH.
  • Investigate the relationship between pulmonary hypertension and pulmonary function in infants diagnosed with congenital diaphragmatic hernia.

Main Methods:

  • Retrospective review of IPFTs and echocardiograms in infants with CDH (2004-2011).
  • Standardization of lung volumes, forced flows, and tidal mechanics using reference values.
  • Linear regression analysis was used to compare infants with and without PH, adjusting for confounders.

Main Results:

  • Infants with CDH and PH showed higher Z-scores for functional residual capacity (FRC), residual volume (RV), FRC/total lung capacity (TLC), and RV/TLC.
  • Lower Z-scores for forced expiratory volume in 0.5 seconds (FEV0.5) and FEV0.5/forced vital capacity were observed in infants with CDH and PH.
  • Persistent PH correlated with higher growth slopes for FRC, RV, and TLC compared to infants without PH.

Conclusions:

  • Infants with CDH and persistent PH experience greater airspace overdistension as they grow.
  • Targeted therapies aimed at improving pulmonary vascular and alveolar formation may enhance care for CDH patients with PH.
Abstract

Keywords:
congenital malformationinfant pulmonary function testingpostnatal lung growth

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