Short-Term Outcome of Neonates with Congenital Heart Disease and Diaphragmatic Hernia Treated with Extracorporeal Membrane Oxygenation

BACKGROUND:

Major congenital heart disease (CHD) is seen in 10% to 15% of patients with congenital diaphragmatic hernia (CDH). Some neonates with CDH and major CHD are treated by surgery and some with extracorporeal membrane oxygenation (ECMO). Because of presumed poor survival, there is significant heterogeneity in management approaches for patients with CDH and associated CHD, and there is no published outcome data of patients who were placed on ECMO support.

METHODS:

To examine outcomes of children with CDH with CHD supported with ECMO, Extracorporeal Life Support Organization (ELSO) registry was queried from 1998 to May 2010. There were a total of 3,342 deployments for CDH and 316 (9.5%) neonates with associated CHD. The median values are as follows: age at presentation, 1 (0 to 52) days; gestational age, 38 (29 to 42) weeks; birth weight, 3 (1.35 to 4.7) kg; admission to time to ECMO, 16 (0 to 1,220) hours; duration of ECMO, 194 (3 to 823) hours; time off of ECMO to extubation, 366 (0 to 7,934) hours; and time off of ECMO to death was 114 (0 to 7,272) hours.

RESULTS:

Initially, ECMO support was venoarterial in 91% (283 of 316), 5 were converted from venovenous to venoarterial ECMO. Overall survival to hospital discharge for all patients with CDH and CHD was 47% (148 of 316). Survival to hospital discharge for hypoplastic left heart syndrome and single-ventricle physiology was 55% (33 of 60), 48% (43 of 89) for ventricular septal defect, and 40% (24 of 60) for coarctation of the aorta patients.

CONCLUSIONS:

Patients with CDH and CHD supported with ECMO have had better than predicted short-term outcomes. In this cohort, overall survival of patient’s with CDH with or without CHD was similar. Patients with single-ventricle physiology had similar short-term outcome to those with 2 ventricle physiology.