Management and outcome of low birth weight neonates with congenital heart disease.

In 100 consecutive neonates with birth weights < or = 2500 gm (range, 540 to 2500 gm; median, 2200 gm), major congenital heart disease (excluding patent ductus arteriosus, isolated atrial septal defect, and ventricular septal defect) was diagnosed between January 1987 and January 1991; 46 had ductus-dependent lesions. Of the 100 neonates, 30 had genetic aberrations or significant associated congenital anomalies. The four most common cardiac diagnoses were tetralogy of Fallot with or without pulmonary atresia (n = 16); coarctation of the aorta (n = 12); transposition of the great arteries (n = 11); and common atrioventricular canal (n = 11). The hospital survival rate for the entire group of 100 neonates was 70%. The patients were separated into three groups on the basis of the time of intervention. Group 1 (early intervention) included 62 infants. These neonates (including 31 with ductus-dependent lesions) had surgical or catheter intervention during the initial hospitalization (median age, 9 days), all at weights < or = 2500 gm. The hospital survival rate was 81% (50/62); survival rates for palliation (78%, 18/23) and for correction (82%, 32/39) were similar. There were 26 neonates in group 2 (late intervention). These neonates did not have surgical intervention during the initial hospitalization. All were managed medically; survivors were discharged and had surgical procedures later (at a median age of 4.3 months). Six neonates (23%) died during medical management; all 20 survivors returned and had surgical procedures, with 90% survival. Overall survival rate for this group was 69% (18/26). The remaining 12 patients (group 3) had complicating features that precluded intervention; none survived. On the basis of these results, we conclude that early intervention, even with corrective surgery, can be performed in low birth weight neonates with an acceptable mortality rate. Prolonged medical therapy to achieve further weight gain did not appear to improve the survival rate.