Semin Perinatol. 2019 Jul 30:151167. doi: 10.1053/j.semperi.2019.07.006. [Epub ahead of print]
https://www.ncbi.nlm.nih.gov/pubmed/31519366
Congenital diaphragmatic hernia-associated pulmonary hypertension.
Gupta VS1, Harting MT2.
Author information
1Department of Pediatric Surgery, McGovern Medical School at the University of Texas Health Science Center at Houston and Children’s Memorial Hermann Hospital, 6431 Fannin St, MSB 5.233, Houston, TX 77030, USA.2Department of Pediatric Surgery, McGovern Medical School at the University of Texas Health Science Center at Houston and Children’s Memorial Hermann Hospital, 6431 Fannin St, MSB 5.233, Houston, TX 77030, USA. Electronic address: matthew.t.harting@uth.tmc.edu.
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
