An evidence-based treatment algorithm for congenital diaphragmatic hernia

Abstract

Background: Congenital diaphragmatic hernia (CDH) affects more than 1100 newborns in the United States each year. Severity of clinical presentation is highly variable. Standardized care improves outcomes by promoting consistency in decision-making and clarifying goals of treatment, but CDH management has not yet been standardized.

Methods: We performed a comprehensive literature review with special consideration for-cardiac dysfunction, indications for extracorporeal membrane oxygenation (ECMO), and timing of repair. In collaboration with experts across specialties, we sought to develop and implement a treatment algorithm based on current CDH literature and our own institutional experience.

Results: Left ventricular (LV) hypoplasia and dysfunction is increasingly recognized as an important contributor to the severity of clinical presentation and cardiac dysfunction seen with CDH. Cardiac dysfunction is associated with poor outcomes and increased mortality. CDH-associated severe hypoxic respiratory failure refractory to medical therapy is one of the most common indications for ECMO in the neonatal period. The decision to initiate ECMO and selection of configuration should be shared by members of a multidisciplinary care team. The optimal timing of repair with respect to ECMO has been evolving in the last 3 decades.

Conclusion: Following our review, we recommend (1) timely and detailed cardiac evaluation with echocardiogram after birth, and (2) early repair on ECMO for high-risk patients and delayed repair post-ECMO for low-risk patients with anticipated short ECMO run. This treatment algorithm is a step toward standardization of CDH management practices, which we expect will improve CDH outcomes at our institution and others.

Keywords: CDH congenital Diaphragmatic hernia; ECMO extracorporeal membrane oxygenation; Level III evidence; left ventricular dysfunction; timing of repair; treatment protocols.