Link: https://www.ncbi.nlm.nih.gov/pubmed/31519366

Semin Perinatol. 2019 Jul 30:151167. doi: 10.1053/j.semperi.2019.07.006. [Epub ahead of print]
Congenital diaphragmatic hernia-associated pulmonary hypertension.
Gupta VS1, Harting MT2.
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Abstract
Congenital diaphragmatic hernia (CDH) is a neonatal pathology in which intrathoracic herniation of abdominal viscera via diaphragmatic defect results in aberrant pulmonary and cardiovascular development. Despite decades of study and many advances in the diagnosis and treatment of CDH, morbidity and mortality remain high, largely due to pulmonary hypertension (PH), along with pulmonary hypoplasia and cardiac dysfunction. In patients with CDH, hypoplastic pulmonary vasculature and alterations in multiple molecular pathways lead to pathophysiologic pulmonary vasculopathy and, for severe CDH, sustained, elevated pulmonary arterial pressures. This review addresses the multiple anatomic and physiologic changes that underlie CDH-associated PH (CDH-PH), along with the multimodal treatment strategies that exist currently and future therapies currently under investigation.

Copyright © 2019. Published by Elsevier Inc.

KEYWORDS:
Cardiac dysfunction; Congenital diaphragmatic hernia; Pulmonary hypertension; Pulmonary vasculature

PMID: 31519366 DOI: 10.1053/j.semperi.2019.07.006