Connor V. Haynes, David H. Stitelman
Yale New Haven Children’s Hospital,
United States
Neoreviews
Neoreviews 2026;
DOI: 10.1542/neo.27-6-021
Abstract
Congenital diaphragmatic hernia (CDH) remains one of the most challenging congenital anomalies encountered by neonatologists and pediatric surgeons, with outcomes largely determined before birth. Although surgical repair of the diaphragmatic defect is essential, postnatal morbidity and mortality are primarily driven by antenatal pulmonary hypoplasia, abnormal pulmonary vascular development, and associated cardiac dysfunction that culminate in respiratory failure and pulmonary hypertension. Advances in prenatal imaging have enabled increasingly refined risk stratification, allowing for the identification of fetuses who are at highest risk for postnatal cardiopulmonary failure. Within this framework, fetal therapies, most notably fetoscopic endoluminal tracheal occlusion, have emerged as the first interventions shown to improve survival in carefully selected cases of severe CDH. However, uncertainty persists regarding optimal patient selection, timing, complications, and the generalizability of outcomes across health care systems. In this review, we examine the pathophysiologic rationale for fetal lung recruitment therapies, contemporary approaches to prenatal diagnosis and severity stratification, and the evidence supporting current fetal interventions. We also discuss how fetal therapy alters postnatal surgical and extracorporeal membrane oxygenation decision-making and highlight emerging prenatal strategies aimed at modifying lung and vascular development before birth. By framing CDH as a prenatal cardiopulmonary disease with postnatal surgical manifestations, we aim to provide a cohesive framework for multidisciplinary counseling, referral, and perioperative planning in the evolving era of fetal therapy.
Category
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
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