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Published on: December 17, 2014
Pulmonary growth and remodeling in infants with high-risk congenital diaphragmatic hernia
D A Beals1, B L Schloo, J P Vacanti
1Department of Surgery, Children's Hospital, Boston, MA 02115.
Insights
Infants with congenital diaphragmatic hernia (CDH) show significant postnatal lung growth and vascular remodeling, leading to larger, less muscular arteries. These changes may reduce pulmonary hypertension over time.
Area of Science:
- Pediatric Pulmonology
- Neonatal Research
- Thoracic Surgery
Background:
- Infants with congenital diaphragmatic hernia (CDH) often experience pulmonary hypoplasia.
- The pattern of postnatal lung development in CDH patients remains largely undocumented.
Purpose of the Study:
- To analyze postnatal pulmonary morphology changes in infants with CDH.
- To understand lung growth patterns and vascular remodeling after CDH.
Main Methods:
- Analysis of lungs from 21 deceased infants with CDH, stratified by age.
- Application of morphometric techniques to assess lung volume, weight, airway generations, alveolar number, and artery muscularization.
Main Results:
- Lung volume and weight increased with age; airway generation number remained constant.
- Radial alveolar number increased, particularly in the contralateral lung.
- Intraacinar artery muscularization decreased with age, with larger arteries becoming less muscular, especially in the contralateral lung.
Conclusions:
- Significant postnatal alveolar growth occurs after CDH repair.
- Postnatal vascular remodeling results in larger, less muscular arteries, potentially decreasing pulmonary arterial hypertension.
- Understanding growth factors could lead to therapies for accelerated pulmonary development in CDH infants.
Abstract:
Infants born with congenital diaphragmatic hernia (CDH) have pulmonary hypoplasia, but the pattern of postnatal growth in these lungs has not been documented. The lungs of 21 children dying with CDH were analyzed to determine how the pulmonary morphology changed with age. The patients were stratified into three age groups for ANOVA analysis (less than 8 days, 8 to 21 days, greater than 21 days). Morphometric techniques previously described were used. Lung volume and weight as well as pulmonary artery length and diameter increased with age (P = .04), whereas the number of airway generations was similar for each group. Radial alveolar number also increased, particularly in the contralateral lung (P = .02). The percentage of intraacinar artery muscularization decreased with age (P = .02), while larger intraacinar arteries showed a nonmuscular structure, again particularly in the contralateral lung (P = .004). It is concluded that: (1) significant lung growth does occur postnatally at the alveolar level after CDH repair; and (2) there is postnatal vascular remodelling resulting in larger and less muscular arteries. These changes should contribute to a decrease in pulmonary arterial hypertension over time. However, the time period over which these changes occur exceeds the current limitations of invasive support measures such as extracorporeal membrane oxygenation. Elucidation of the factors responsible for this growth could result in new therapeutic strategies to enhance or accelerate postnatal pulmonary development in infants with CDH.
