Shazia Bombal, Neil Patel
Stanford University and Lucille Packard Children’s Hospital. Royal Hospital for Children, Glasgow.
United States and United Kingdom
Seminars in Fetal and Neonatal Medicine
Semin Fetal Neonatal Med 2022; 27:
DOI: 10.1016/j.siny.2022.101383
Abstract
Congenital diaphragmatic hernia (CDH) contributes to neonatal morbidity and mortality worldwide. Pulmonary hypertension (PH) is a key component of CDH pathophysiology and critical consideration for management and therapeutic options. PH associated with CDH has traditionally been attributed to pulmonary vascular maldevelopment and associated lung parenchymal hypoplasia, leading to pre-capillary increase in pulmonary vascular resistance (PVR). However, there is increasing recognition that left ventricular hypoplasia, dysfunction and elevated end diastolic pressure may contribute to post-capillary pulmonary hypertension in CDH patients.
The interplay of these mechanisms and associated dysfunction in the right and left ventricles results in variable hemodynamic phenotypes in CDH. Clinical assessment of individual phenotype may help guide personalized management strategies, including effective use of pulmonary vasodilators and extra-corporeal membrane oxygenation. Ongoing investigation of the underlying mechanisms of PH in CDH, and efficacy of physiology-based treatment approaches may support improvement in outcomes in this challenging condition.
Category
Review Articles Concerning Pulmonary Vascular Disease
Class 3. Pulmonary Vascular Disease Associated with Lung Hypoplasia
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
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