This study aimed to determine the prevalence of congenital heart defects (CHD) in metropolitan Atlanta from 1998 to 2005 using current diagnostic standards. The Metropolitan Atlanta Congenital Defects Program (MACDP), a population-based birth defects surveillance system, was utilized to identify infants with CHD. Physiological shunts and those associated with prematurity were excluded. The study found an overall prevalence of 81.4 CHD cases per 10,000 births, with the most common types being muscular ventricular septal defect (27.5/10,000), perimembranous ventricular septal defect (10.6/10,000), and secundum atrial septal defect (10.3/10,000). Tetralogy of Fallot, the most common cyanotic CHD, had a prevalence twice that of transposition of the great arteries (4.7 vs. 2.3/10,000). Maternal age and multiple-gestation pregnancies were associated with certain CHD types, and several defects were more common in girls. The study provides current prevalence estimates for various CHD subtypes, which can be used to assess variations in prevalence across populations, time, or space.This study aimed to determine the prevalence of congenital heart defects (CHD) in metropolitan Atlanta from 1998 to 2005 using current diagnostic standards. The Metropolitan Atlanta Congenital Defects Program (MACDP), a population-based birth defects surveillance system, was utilized to identify infants with CHD. Physiological shunts and those associated with prematurity were excluded. The study found an overall prevalence of 81.4 CHD cases per 10,000 births, with the most common types being muscular ventricular septal defect (27.5/10,000), perimembranous ventricular septal defect (10.6/10,000), and secundum atrial septal defect (10.3/10,000). Tetralogy of Fallot, the most common cyanotic CHD, had a prevalence twice that of transposition of the great arteries (4.7 vs. 2.3/10,000). Maternal age and multiple-gestation pregnancies were associated with certain CHD types, and several defects were more common in girls. The study provides current prevalence estimates for various CHD subtypes, which can be used to assess variations in prevalence across populations, time, or space.