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Published on: August 15, 2022
Outcomes of congenital diaphragmatic hernia repair on extracorporeal life support
Jamie Golden1, Nicole Jones1, Jessica Zagory1
1Division of Pediatric Surgery, Children's Hospital Los Angeles, 4650 Sunset Blvd, Mailstop 100, Los Angeles, CA, 90027, USA.
Insights
Repairing congenital diaphragmatic hernia (CDH) after extracorporeal life support (ECLS) decannulation improves survival. Infants repaired on ECLS had higher mortality and bleeding rates, underscoring the benefit of post-decannulation repair for CDH patients.
Area of Science:
- Neonatal Surgery
- Pediatric Critical Care
- Extracorporeal Life Support (ECLS)
Background:
- Congenital diaphragmatic hernia (CDH) is a severe condition often requiring extracorporeal life support (ECLS) for refractory pulmonary hypertension.
- The optimal timing for surgical repair in CDH patients supported by ECLS remains a critical clinical question.
Purpose of the Study:
- To evaluate single-center outcomes of infants with CDH treated with ECLS.
- To compare the utility of surgical repair performed on ECLS versus repair after decannulation in CDH patients.
Main Methods:
- Retrospective review of infant records diagnosed with CDH between 2004 and 2014.
- Analysis of outcomes for infants undergoing ECLS, stratified by repair timing (on ECLS vs. post-decannulation).
Main Results:
- Of 177 CDH infants, 66 (37%) received ECLS. Repair was delayed in patients on ECLS (19 vs. 10 days).
- Infants repaired on ECLS experienced longer ECLS runs (22 vs. 12 days) and higher rates of bleeding and mortality.
- Survival rates were 36% for repair on ECLS, 85% for repair post-decannulation, and 54% overall for ECLS patients.
Conclusions:
- Surgical repair of CDH post-decannulation is associated with excellent outcomes and no repair-related mortality.
- Repairing CDH while on ECLS is linked to the lowest survival rates, with significant mortality attributed to surgical bleeding.
Purpose:
Extracorporeal life support (ECLS) is applied to refractory pulmonary hypertension in congenital diaphragmatic hernia (CDH). We evaluate the single-center outcomes of infants with CDH to determine the utility of late repair on ECLS versus repair post-decannulation.
Methods:
Records of infants with CDH (2004-2014) were retrospectively reviewed.
Results:
CDH was diagnosed in 177 infants. Sixty six (37%) underwent ECLS, of which, 11 died prior to repair, 33 were repaired post-decannulation, and 22 were repaired on ECLS. Repair was delayed in patients on ECLS (19 versus 10 days, p < 0.001). Patients repaired on ECLS had longer ECLS runs (22 versus 12 days, p < 0.001) and higher rates of bleeding and mortality than those repaired post-decannulation. Survival was 54% in infants undergoing ECLS, 65% in those who underwent repair, 36% in those repaired during ECLS, and 85% in those who were decannulated prior to repair. Eighteen percent (N = 4) of deaths after repair on ECLS were attributable to surgical bleeding. The remainder was due to pulmonary hypertension or sepsis.
Conclusion:
Infants who underwent CDH repair post-decannulation had excellent outcomes and no mortalities attributable to repair. Neonates who underwent repair on ECLS late on bypass had the lowest survival rate with only 18% of mortality in this cohort attributable to surgical bleeding.

