Improved survival in congenital diaphragmatic hernia with evolving therapeutic strategies.

OBJECTIVE

To compare the survival rates for 3 therapeutic eras, each using different treatment strategies for the management of newborns with congenital diaphragmatic hernia (CDH).

DESIGN

Retrospective review of all infants with CDH from 1970 through 1997.

SETTING

Tertiary care children's hospital.

PARTICIPANTS

A total of 203 newborns with CDH.

INTERVENTIONS

Extracorporeal membrane oxygenation (ECMO) was performed with arterial and venous cannulation connected to a membrane oxygenatorroller pump perfusion apparatus, using systemic heparinization. Delayed operative therapy involved operative repair 2 to 5 days after birth using preoperative ventilation support only. Since 1970, 203 newborns with CDH were managed in 3 therapeutic eras: era 1 (1970-1983, 102 patients) was immediate CDH repair with postoperative ventilator and pharmacologic support; era 2 (1984-1988, 45 patients) was immediate repair with postoperative ventilator support (18 patients), immediate ECMO with CDH repair on ECMO (4 patients), or immediate repair with postoperative ECMO (23 patients); and era 3 (1989-1997, 56 patients) was immediate ECMO with repair on ECMO (23 patients), immediate repair with postoperative ECMO (9 patients), or delayed (2-5 days) CDH repair (24 patients).

MAIN OUTCOME MEASURES

Survival, defined as discharge from the hospital, and morbidity.

RESULTS

Survival was 42% (43/102 patients) in era 1, 58% (26/45 patients) in era 2, and 79% (44/56 patients) in era 3 (P<.02 vs eras 1 and 2). In era 3, the survival for immediate ECMO with repair on ECMO was 57% (13/23 patients), 89% (8/9 patients) for immediate repair with postoperative ECMO, and 96% (23/24 patients) for delayed repair. Eight late deaths were caused by pulmonary hypertension (1 death), sudden infant death syndrome (1 death), and other causes (6 deaths). Morbidity in survivors included mild neurologic deficit (5 patients) and pulmonary disease (3 patients).

CONCLUSION

These data demonstrate a significant improvement in survival in CDH with preoperative ECMO and with delayed repair with and without ECMO support and suggest that immediate repair of CDH without the availability of ECMO support should be abandoned.