Cardiac Adaptation to Severe Congenital Diaphragmatic Hernia

ABSTRACT

Objective Prenatal heart adaptations to congenital diaphragmatic hernia (CDH) could help define postnatal outcome. Methods We retrospectively analyzed post-mortem tissues from fetuses with severe CDH (n = 7). Histology and immunohistochemical distribution of desmin, muscle actin [HHF35], endothelin-1 [ET-1] and TGF-β were evaluated. Results In the atrium, desmin, HHF35, ET-1, TGF-β were found expressed only in preterm CDH. Dishomogeneous ventricular distribution of cardiac growth factors were detected in term CDH. The cardiomyocyte nucleus/cytoplasmatic ratio in CDH was higher compared with controls (p = 0.01). Small intramyocardial artery density and vascular wall thickness was increased in CDH compared with controls (p = 0.03 and p < 0.01). In comparison with the ventricles, the interventricular septum showed a greater vessel density (p = 0.01) and a greater vascular wall thickness, particularly compared with the CDH right ventricle (p = 0.02). Conclusion Left ventricle immaturity seems to be a cardiac adaptive response of severe CDH in utero.

Keywords:

• fetal
• heart
• congenital diaphragmatic hernia
• growth
• remodeling

Acknowledgments

We wish to acknowledge the “Associazione Bambini con la CCAM” for supporting research in the University of Pavia, Pediatric Surgery Unit.

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.