Semin Pediatr Surg

. 2023 Oct 17;32(4):151328.

 doi: 10.1016/j.sempedsurg.2023.151328. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/37939639/

Extracorporeal life support in congenital diaphragmatic hernia

Kylie I Holden ¹, Alice M Martino ², Yigit S Guner ², Matthew T Harting ³

Affiliations expand

• PMID: 37939639
• DOI: 10.1016/j.sempedsurg.2023.151328

Abstract

Congenital diaphragmatic hernia (CDH) is the most common indication for ECLS in neonatal respiratory failure. The ultimate purpose of ECLS is to grant cardiopulmonary support, allowing time for operative intervention and optimization of cardiopulmonary function as the pathophysiologic processes of pulmonary hypertension, pulmonary hypoplasia, and ventricular dysfunction either improve or resolve. In CDH, ECLS plays a crucial role in the management of the most challenging patients, facilitating postnatal stabilization, allowing a ventilation strategy which minimizes barotrauma and volutrauma, and permitting treatment of and recovery from pulmonary hypertension and/or cardiac dysfunction. Understanding the nuances of CDH patients, which differ from other forms of neonatal respiratory failure, and the benefits of ECLS for these infants, is crucial for effective management. CDH patients present distinct challenges. Every aspect of ECLS, from mode of support and anticoagulation medication to pump selection, ventilation strategy, pulmonary hypertension management, and the weaning process, requires meticulous consideration. ECLS for CDH serves as a bridge to making informed decisions, granting clinicians stability and time to manage / recover from specific pathophysiologic consequences, and it offers the potential for survival among even the most challenging and complex patients. As overall care and management for infants with CDH receiving ECLS continue to improve, the focus has shifted toward managing survivor morbidity. Given the multisystem nature of the disease, this requires significant experience, expertise, and multidisciplinary teamwork to optimize long-term outcomes for these patients.

Keywords: CDH surgical repair; Congenital diaphragmatic hernia (CDH); Diaphragm; Extracorporeal life support (ECLS); Long-term outcomes.

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