Sandy Johng, Maria V. Fraga, Neil Patel, Florian Kipfmueller, Abhijit Bhattacharya, Shazia Bhombal
Children’s Hospital of Philadelphia and Perelman School of Medicine. Royal Hospital for Children. Children’s Hospital and University of Bonn. Emory University and Children’s Healthcare of Atlanta.
United States, United Kingdom and Germany
Neoreviews
Neoreviews 2023; 24(11):e720-e732
DOI: 10.1542/neo.24-11-e720
Abstract
Congenital diaphragmatic hernia (CDH) results in abdominal contents entering the thoracic cavity, affecting both cardiac and pulmonary development. Maldevelopment of the pulmonary vasculature occurs within both the ipsilateral lung and the contralateral lung. The resultant bilateral pulmonary hypoplasia and associated pulmonary hypertension are important components of the pathophysiology of this disease that affect outcomes. Despite prenatal referral to specialized high-volume centers, advanced ventilation strategies, pulmonary hypertension management, and the option of extracorporeal membrane oxygenation, overall CDH mortality remains between 25% and 30%. With increasing recognition that cardiac dysfunction plays a large role in morbidity and mortality in patients with CDH, it becomes imperative to understand the different clinical phenotypes, thus allowing for individual patient-directed therapies. Further research into therapeutic interventions that address the cardiopulmonary interactions in patients with CDH may lead to improved morbidity and mortality outcomes.
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: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No