Purpose Centers that care for newborns with Congenital Diaphragmatic Hernia (CDH) may impose selection criteria for offering or limiting aggressive support in those patients most severely affected. The purpose of this study was to analyze outcomes in newborns with highly severe CDH uniformly treated for survival. Methods We reviewed 172 consecutive inborn patients without associated lethal anomalies treated at a single institution with a dedicated CDH program. Survival, respiratory outcome, and time to discharge in the most severe 10% (or fewer) of patients based on the physiologic measures of 5-minute Apgar, CDH Study Group (CDHSG) predicted survival, need for ECMO in the first 6 hours, and need for ECMO in the first 3 hours of life were studied. We also identified patients with best PaCO2 greater than 100 and best pH less than 7.0. A multivariate model (AUC-0.92) predicting mortality was also used to define the most severe 10%. Results Of 172 consecutive inborn patients, 18 had a 5-minute Apgar of 3 or less, and 11 survived (61%), 10 had a 5-minute Apgar of 2 or less, and 6 survived (60%), and 6 had a 5-minute Apgar of 1 or less, and 4 survived (67%). Seventeen had a CDHSG predicted survival less than 25%, and 9 survived (53%). Thirteen of 172 required ECMO for rescue in the first 6 hours of life, and 9 survived (69%), including 7 in the first 3 hours, and 5 survived (71%). Despite focused resuscitation in the delivery room and high levels of ventilatory support, 22 patients had a best PCO2 greater than 100 and best pH less than 7.0 for 1 hour or longer. Twelve of these 22 survived to discharge (55%). Of 17 defined by multivariate predictive model as the most severe, 8 survived (47%) with zero of the 3 ECMO ineligible prematures surviving. Of the 16 (10%) most severe ECMO-eligible patients, 10 of 16 survived (63%). All survivors were discharged home on no ventilatory support greater than nasal cannula oxygen. Conclusion In newborn CDH patients without lethal associated anomalies, accepted measures of physiologic severity failed to predict mortality. Survival met or exceeded 50% even in the most severe 10% as defined by these measures. These data support the practice of treating each patient for survival regardless of the physiologic severity in the first hours of life, and selection criteria for not offering ECMO should be re-evaluated where practiced.

Background Congenital diaphragmatic hernia (CDH) is a frequently lethal birth defect and despite advances, extracorporeal life-support (ECMO) is commonly required for severely affected patients. Published data suggest that CDH survival after 2 weeks on ECMO is poor. Many centers limit duration of ECMO support. Study Design A single institution retrospective review of 19 years of CDH patients treated with ECMO, designed to evaluate which factors affect survival and duration of ECMO, and to define how long patients should be supported. Results Of two hundred and forty consecutive CDH patients without lethal associated anomalies, 96 were treated with ECMO and 72 (75%) survived. Eighty required a single run of ECMO and 65 survived (81%), 16 required a second ECMO run and 7 survived (44%). Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO survival had dropped to 15%, and after 40 days of ECMO support there were no survivors. Apgar-1, Apgar-5, and CDH Study Group Predicted Survival all correlated with survival on ECMO, need for second ECMO, and duration of ECMO. LHR also correlated with duration of ECMO. All survivors were discharged breathing spontaneously with no support other than nasal cannula oxygen if needed. Conclusions In patients with severe CDH, improvement in pulmonary function sufficient to wean from ECMO may take 4 weeks or longer, and may require a second ECMO run. Pulmonary outcomes in these CDH patients can still be excellent, and the assignment of arbitrary ECMO treatment durations less than 4 weeks should be avoided.

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Objective To assess the impact of varying approaches to CDH repair timing on survival and need for ECMO when controlled for anatomic and physiologic disease severity in a large consecutive series of CDH patients. Summary Background Data Our publication of 60 consecutive CDH patients in 1999 showed that survival is significantly improved by limiting lung inflation pressures and eliminating hyperventilation. Methods We retrospectively reviewed 268 consecutive CDH patients, combining 208 new patients with the 60 previously reported. Management and ventilator strategy were highly consistent throughout. Varying approaches to surgical timing were applied as the series matured. Results Patients with anatomically less-severe left liver-down CDH had significantly increased need for ECMO if repaired in the first 48 hours, while patients with more-severe left liver-up CDH survived at a higher rate when repair was performed before ECMO. Overall survival of 268 patients was 78%. For those without lethal associated anomalies, survival was 88%. Of these, 99% of left liver-down CDH survived, 91% of right CDH survived. and 76% of left liver-up CDH survived. Conclusions This study shows that patients with anatomically less severe CDH benefit from delayed surgery while patients with anatomically more severe CDH may benefit from a more aggressive surgical approach. These findings show that patients respond differently across the CDH anatomic severity spectrum, and lay the foundation for the development of risk specific treatment protocols for patients with CDH.