Clinical characteristics and outcomes of patients with cardiac defects and congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the impact of associated heart defects on outcomes to discharge, and identify factors affecting survival of all infants born with congenital diaphragmatic hernia (CDH) in last decade using Congenital Diaphragmatic Hernia Study Group data.

STUDY DESIGN: This was a retrospective review of all infants with CDH enrolled in Congenital Diaphragmatic Hernia Study Group database from January 2000 to December 2010. The study cohort was divided into 3 groups (GRP): GRP 1, CDH with major heart defects; GRP 2, CDH with minor heart defects; and GRP 3, CDH with no reported heart defects.

RESULTS: The 4268 enrolled infants included 345 (8%) in GRP 1, 412 (10%) in GRP 2, and 3511 (82%) in GRP 3. Survival was significantly lower in GRP 1 compared with GRP 2 and GRP 3 (36% vs 73%). In GRP 1, the most common defects were left heart obstructive lesions (34%). Survival was lowest in infants with transposition of great arteries (0%) and single ventricle physiology (16%). There was no change in survival rate for any group between 2000-2005 and 2006-2010. In GRP 1, factors that predicted lower survival were birth weight <2.5 kg, associated noncardiac anomalies, single ventricle physiology, no sildenafil therapy, no CDH repair, and no cardiac repair.

CONCLUSION: Survival is significantly lower in patients with CDH and major heart defects compared with patients with minor or no heart defects. Outcomes of newborns with CDH and major heart defects have not improved over the last decade.