Detrimental effects of standard medical therapy in congenital diaphragmatic hernia

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.