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An evidence-based treatment algorithm for congenital diaphragmatic hernia
Latoya A Stewart1, Yeu Sanz Wu2, Alexandra Channing3
1Columbia University Vagelos College of Physicians and Surgeons, New York, NY, USA.
Insights
Standardizing congenital diaphragmatic hernia (CDH) care is crucial. A new algorithm recommends early cardiac evaluation and tailored surgical repair timing based on patient risk, aiming to improve outcomes for newborns with CDH.
Area of Science:
- Neonatal Surgery
- Pediatric Cardiology
- Critical Care Medicine
Background:
- Congenital diaphragmatic hernia (CDH) impacts over 1100 US newborns annually with variable severity.
- Current CDH management lacks standardization, hindering consistent decision-making and treatment goal clarity.
- Standardized care protocols are known to improve patient outcomes in complex conditions.
Purpose of the Study:
- To develop and implement a standardized treatment algorithm for congenital diaphragmatic hernia (CDH).
- To address variability in CDH clinical presentation and management.
- To improve outcomes for newborns diagnosed with CDH.
Main Methods:
- Comprehensive literature review focusing on cardiac dysfunction, ECMO indications, and repair timing.
- Collaborative development of a treatment algorithm with multidisciplinary experts.
- Integration of institutional experience with current CDH literature.
Main Results:
- Left ventricular (LV) hypoplasia and dysfunction significantly contribute to CDH severity and poor outcomes.
- Severe hypoxic respiratory failure refractory to medical therapy is a primary indication for neonatal extracorporeal membrane oxygenation (ECMO).
- Optimal surgical repair timing relative to ECMO remains an evolving aspect of CDH management.
Conclusions:
- Recommend prompt echocardiogram for cardiac evaluation post-birth in CDH cases.
- Advocate for early ECMO repair in high-risk CDH infants and delayed post-ECMO repair in low-risk infants.
- The developed algorithm represents a step towards standardizing CDH care to enhance patient outcomes.
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.
