Journal
SEMINARS IN PERINATOLOGY
Volume 44, Issue 1, Pages -Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1053/j.semperi.2019.07.006
Keywords
Congenital diaphragmatic hernia; Pulmonary hypertension; Pulmonary vasculature; Cardiac dysfunction
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Funding
- Center for Clinical and Translational Sciences Training Award, University of Texas McGovern Medical School [5KL2-TR00037010]
- Ladybug foundation award
- Men of Distinction award
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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. (C) 2019 Published by Elsevier Inc.
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