Distribution of and mortality from serious congenital heart disease in very low birth weight infants.

OBJECTIVE

To characterize serious congenital heart disease in very low birth weight (VLBW) infants (born at <1500 g or a gestational age of 22-29 weeks) in a large, international database.

PATIENTS AND

METHODS

We analyzed a database of 99 786 VLBW infants born or treated at 703 NICUs between calendar years 2006 and 2007. We defined serious congenital heart disease as 1 of 14 specific lesions or any other structural congenital heart disease that required surgical or medical treatment by initial hospital discharge or by the age of 1 year. We reviewed records for all infants with cardiac diagnoses and other genetic syndromes and associations to determine which had serious congenital heart disease. We excluded nonstructural disease as well as isolated and untreated atrial or ventricular septal defects. We determined the frequency of serious congenital heart disease, compared overall mortality rates of those with and without serious congenital heart disease, and determined the distribution of specific lesions and mortality for each diagnosis.

RESULTS

Of 99 786 VLBW infants studied, 893 had serious congenital heart disease (8.9 per 1000). The most common lesions were tetralogy of Fallot (n = 166 [18.6% of those with serious congenital heart disease]), aortic coarctation (n = 103 [11.5%]), complete atrioventricular canal (n = 81 [9.1%]), pulmonary atresia (n = 73 [8.2%]), and double-outlet right ventricle (n = 68 [7.6%]). The mortality rate of those with serious congenital heart disease was 44%, compared with 12.7% in those without serious congenital heart disease (P < .0001).

CONCLUSIONS

Serious congenital heart disease is probably more frequent in VLBW infants treated in NICUs than in the general live-born population, and the distribution reflects lesions associated with extracardiac malformations. VLBW infants with serious congenital heart disease have higher a mortality rate than those without, independent of other risk factors.