Objective To evaluate the impact of a nonstandard ventilation strategy on s
urvival in congenital diaphragmatic hernia (CDH).
Background
Despite recent advances, including nitric oxide, CDH remains an unsolved pr
oblem with a mortality rate of 35% to 50%. Hyperventilation and alkalizatio
n remain common therapies.
Methods
In 1992, the authors prospectively abandoned hyperventilation and alkalizat
ion. Patients are lightly sedated and ventilated with the lowest pressure p
roviding adequate chest movement, and the rate is set to patient comfort. N
itric oxide and extracorporeal membrane oxygenation (ECMO) are reserved for
life-threatening instability. Surgical repair is delayed 1 to 5 days. Sixt
y consecutive patients are compared with 29 previous patients treated with
hyperventilation and alkalization, 13 before and 16 after the availability
of ECMO.
Results
Overall, 47 of 60 patients (78%) in study era 3 survived compared with 2 of
13 (15%) in the hyperventilation era and 7 of 16 (44%) in the hyperventila
tion/ECMO era (p < 0.0001). The disease severity and the incidence of assoc
iated anomalies did not differ between groups. To compare management strate
gies, patients who had treatment withheld because of lethal associated cond
itions were then removed from analysis, Peak inspiratory pressure and arter
ial pH were lower (p < 0.0001) and Pace, was higher (p < 0.05) in era 3 tha
n in the previous eras. The rate of pneumothorax (1.9%) decreased (p < 0.00
01), In era 3, survival was 47 of 53 (89%) treated patients, and 23 of 25 i
nborn patients with isolated CDH survived (92%).
Conclusions
Nonstandard ventilatory support of patients with CDH has led to significant
ly improved survival rates. This study sets a survival benchmark and strong
ly suggests the negative effects of hyperventilation and alkalization.