Deepika Sankaran. Satyan Lakshminrusimha, Michelle J. Lim
University of California, Davis.
United States
Seminars in Fetan and Neonatal Medicine
Semin Fetal Neonatal Med 2025;
DOI: 10.1016/j.siny.2025.101649
Abstract
Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly that occurs due to incomplete closure of the diaphragm followed by herniation of abdominal contents into the chest. Fetal hemodynamics are altered in CDH due to pulmonary alveolar and vascular hypoplasia, low pulmonary venous return and in some cases, hypoplasia or dysfunction of the left heart. CDH is associated with high risk of morbidity and mortality due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn secondary to pulmonary vascular hypoplasia and remodeling. Resuscitation in the delivery room involves optimizing cardiopulmonary transition from fetal to neonatal circulation through gentle ventilation, endotracheal intubation and gastric decompression. Infants with CDH often present with hypoxemic respiratory failure in the immediate postnatal period due to pulmonary hypertension. The understanding of critical cardiopulmonary interactions and the distinguishing features of CDH sub-phenotypes and degree of cardiac involvement may aid in an augmented precision-based approach to invasive ventilation, vasoactive use, and ECMO management. Improved survival is reported after fetal in-utero interventions such as fetoscopic endoluminal tracheal occlusion (FETO) with CDH and severe secondary pulmonary hypoplasia. Clinical outcomes can potentially be improved by optimizing cardiopulmonary transition in the delivery room and cardiopulmonary interactions in the immediate the postnatal period.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No