Objective.  Review of the prevalence of congenital heart defects (CHD) and fetal alcohol spectrum disorder (FASD).

Design.  We conducted a search of the Medline and Pubmed databases to identify papers reporting the association. We then searched the reference lists of the papers and reference books for additional sources.

Results.  We found 29 studies that met our inclusion criteria. In the 12 case series studies of subjects with FASD, the proportion of cases with a CHD (atrial [ASD] and ventricular [VSD] septal defects, other defects, or unspecified CHD) ranged from 33% to 100%. From the 14 retrospective studies, the rate of septal defects was 21%, other structural defects 6% and unspecified defects was 12%. For the 2 case–control studies, the odds of CHD ranged from 1.0 (subjects with fetal alcohol effect) to 18.0 (subjects with fetal alcohol syndrome). In the 1 prospective study of CHD the OR for a child to have CHD and FASD was 1.0.

Key Conclusion.  Pediatric cardiologists may have frequent contact with children with FASD and increased levels of attention to prenatal alcohol exposure as a potential etiology of CHD is indicated.