Congenital diaphragmatic hernia (CDH) is a condition that affects the development of the diaphragm and the maturation of the cardiopulmonary system.1 It involves the abnormal embryologic development of the diaphragm and subsequent compression of the cardiopulmonary system resulting from herniation of abdominal organs. The severity of CDH can vary, with individuals who have additional anomalies, larger defects, or receive extracorporeal life support (ECLS) posing the greatest challenges in terms of resources and, ultimately, bear the majority of the morbidity and mortality.

ECLS is utilized in the setting of compromised cardiopulmonary function where there is inadequate organ perfusion, delivery of oxygen, or removal of carbon dioxide. It is appropriate for individuals who have reversible, insufficient pulmonary function to adequately oxygenate and ventilate, or reversible, inadequate cardiac function for end organ perfusion. ECLS operates by pumping the circulating blood through an artificial lung, oxygenating it, removing carbon dioxide, and then returning it to the body. Evidence suggests that CDH patients benefit from ECLS support, particularly the highest risk patients, supported in high-quality, high-volume ECLS centers.2 Unfortunately, approximately half of the infants who receive ECLS for CDH do not survive, and those who do face an increased risk of long-term health issues such as neurodevelopment delay, gastrointestinal morbidity, and ongoing respiratory challenges.3 Therefore, opportunities for optimization exist.

There is significant variation in the approaches and practices among healthcare institutions regarding CDH management, including criteria for initiating ECLS, methods of support, bedside care strategies, timing and approaches for surgical repair, and liberation from support.4, 5, 6 This wide variability highlights the opportunity for evidence-based alignment and standardization of care. This review is designed to offer evidence-based strategies and encourage uniform care practices for these complex patients, fostering alignment among healthcare providers and ultimately enhancing outcomes.