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.