Outcomes in the physiologically most severe congenital diaphragmatic hernia (CDH) patients: Whom should we treat?

Abstract

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.

Role of ECMO in congenital diaphragmatic hernia

“…The difficulty with predicting the need for and outcome of ECMO in CDH is identifying neonates with potentially reversible disease, compared with those that are irreversible from the outset 23. ECMO in neonates with PPHN associated with other more reversible lung disease carries a much better outcome than in CDH 24.…”

Resuscitation of infants with congenital diaphragmatic hernia

“…A low Apgar score (2 or less) at 5 min was reported to be associated with 60% survival to discharge 8. Respiratory function monitoring is now available for use in the labour suite 9.…”

Extracorporeal Membrane Oxygenation for Neonates with Congenital Renal and Urological Anomalies and Pulmonary Hypoplasia: A Case Report and Review of the Extracorporeal Life Support Organization Registry

“…[15][16][17] Kays et al have recently reported that after multivariate modeling of severity to define the worst 10% for CDH, survival in this cohort approached 50%. 18 They also reported mean time to discharge in survivors to be 3.25 months, indicating these children require a prolonged hospital course. It remains unclear how to risk stratify the degree of pulmonary hypoplasia to determine long-term survival or need for ECMO support in this more well-studied patient population 9,19 .…”