Arch Dis Child Fetal Neonatal Ed

. 2021 Dec 24;fetalneonatal-2021-322617. doi: 10.1136/archdischild-2021-322617. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/34952853/

Neonatal and fetal therapy of congenital diaphragmatic hernia-related pulmonary hypertension

Felix R De Bie ^1 2, Catherine M Avitabile ^3 4, Luc Joyeux ², Holly L Hedrick ^5 4, Francesca M Russo ^2 6, David Basurto ², Jan Deprest ^2 7, Natalie E Rintoul ^4 8Affiliations expand

• PMID: 34952853
• DOI: 10.1136/archdischild-2021-322617

Abstract

Congenital diaphragmatic hernia (CDH) is a complex malformation characterised by a triad of pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. Much of the mortality and morbidity in CDH is largely accounted for by PH, especially when persistent beyond the neonatal period and refractory to available treatment. Gentle ventilation, haemodynamic optimisation and pulmonary vasodilation constitute the foundations of neonatal treatment of CDH-related PH (CDH-PH). Moreover, early prenatal diagnosis, the ability to assess severity and the developmental nature of the condition generate the perfect rationale for fetal therapy. Shortcomings of currently available clinical therapies in combination with increased understanding of CDH pathophysiology have spurred experimental drug trials, exploring new therapeutic mechanisms to tackle CDH-PH. We herein discuss clinically available neonatal and fetal therapies specifically targeting CDH-PH and review the most promising experimental treatments and future research avenues.

Keywords: intensive care units; neonatal; neonatology.

© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.