Contemporary outcomes in infants with congenital heart disease and bochdalek diaphragmatic hernia

Abstract

Background: Fifteen percent of infants with congenital diaphragmatic hernia (CDH) are born with a coexisting cardiac anomaly. The purpose of this study was to evaluate contemporary outcomes in this patient population and to identify potential risk factors for in-hospital mortality.

Methods: Data from all CDH neonates with congenital heart disease managed at a single pediatric tertiary care referral center between 1997 and 2011 were retrospectively analyzed.

Results: Forty (18%) of 216 CDH patients had a cardiac anomaly. This group was associated with a significant decrease in overall survival when compared with patients without cardiac anomaly (55% versus 81%; p = 0.001). There was no association between type of cardiac anomaly and mortality based on risk stratification according to the Risk Adjustment for Congenital Heart Surgery and The Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery scoring systems (p = 0.86 and p = 0.87, respectively). Birth weight was similarly no different between survivors and nonsurvivors (2.8 ± 0.6 kg versus 2.8 ± 0.9 kg, respectively; p = 0.98). There was a trend toward increased extracorporeal membrane oxygenation use among nonsurvivors (p = 0.13). Infants with hemodynamic stability enabling subsequent cardiac repair were associated with lower mortality (p = 0.04). Survivors had a wide spectrum of long-term morbidity, but most had some evidence of neurodevelopmental impairment.

Conclusions: This large single-institution series suggests that the overall prognosis of infants with concomitant CDH and congenital heart disease can be quite variable, regardless of the type of heart anomaly. Hemodynamic instability and need for extracorporeal membrane oxygenation correlate with higher mortality. Although some long-term survivors have excellent outcomes, most suffer from chronic, long-term morbidities.