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. Author manuscript; available in PMC: 2022 Mar 11.
Published in final edited form as: Birth Defects Res. 2022 Jan 19;114(2):45–56. doi: 10.1002/bdr2.1980

TABLE 2.

Critical congenital heart defect (CCHD) counts and prevalence (per 10,000 live births) for 19 U.S. population-based surveillance programs,a 2014-2018

Critical Congenital Heart Defects (CCHDs)b
 Coarctation of aorta
 Common truncus (truncus arteriosus or TA)
 Dextro-Transposition of great arteries (d-TGA)
 Double outlet right ventricle (DORV)
 Ebstein anomaly
 Hypoplastic left heart syndrome
Variable values Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe
Total 18,587 19.6 19.3-19.9 5,464 5.8 5.6-5.9 557 0.6 0.5-0.6 2,201 2.3 2.2-2.4 2,183 2.3 2.2-2.4 713 0.8 0.7-0.8 2,546 2.7 2.6-2.8
Case ascertainment methodologya
Active case finding 8,835 20.1 19.7-20.6 2,375 5.4 5.2-5.6 277 0.6 0.6-0.7 1,078 2.5 2.3-2.6 1,038 2.4 2.2-2.5 319 0.7 0.6-0.8 1,077 2.5 2.3-2.6
Passive case finding 9,752 19.2 18.8-19.6 3,089 6.1 5.9-6.3 280 0.6 0.5-0.6 1,123 2.2 2.1-2.3 1,145 2.3 2.1-2.4 394 0.8 0.7-0.9 1,469 2.9 2.7-3.0
Maternal race/ethnicity f
White, Non-Hispanic 9,034 19.8 19.4-20.2 2,912 6.4 6.2-6.6 251 0.6 0.5-0.6 1,200 2.6 2.5-2.8 952 2.1 2.0-2.2 329 0.7 0.6-0.8 1,276 2.8 2.6-3.0
Black, Non-Hispanic 3,016 19.9 19.2-20.7 750 5.0 4.6-5.3 87 0.6 0.5-0.7 312 2.1 1.8-2.3 420 2.8 2.5-3.1 68 0.4 0.3-0.6 472 3.1 2.8-3.4
Hispanic 5,105 19.7 19.1-20.2 1,458 5.6 5.3-5.9 170 0.7 0.6-0.8 554 2.1 2.0-2.3 634 2.4 2.3-2.6 244 0.9 0.8-1.1 630 2.4 2.2-2.6
Asian or Pacific Islander, Non-Hispanic 891 16.3 15.2-17.4 214 3.9 3.4-4.5 28 0.5 0.3-0.7 86 1.6 1.3-1.9 114 2.1 1.7-2.5 47 0.9 0.6-1.1 80 1.5 1.2-1.8
American Indian or Alaska Native, Non-Hispanic 148 28.3 23.9-33.2 35 6.7 4.7-9.3 5 1.0 0.3-2.2 9 1.7 0.8-3.3 17 3.2 1.9-5.2 7 1.3 0.5-2.8 15 2.9 1.6-4.7
Maternal age (years) f
<35 14,744 18.8 18.5-19.1 4,335 5.5 5.4-5.7 442 0.6 0.5-0.6 1,815 2.3 2.2-2.4 1,726 2.2 2.1-2.3 565 0.7 0.7-0.8 2,079 2.7 2.5-2.8
35+ 3,751 23.1 22.4-23.9 1,118 6.9 6.5-7.3 113 0.7 0.6-0.8 377 2.3 2.1-2.6 444 2.7 2.5-3.0 146 0.9 0.8-1.1 447 2.8 2.5-3.0
Infant sex f
Male 10,594 21.9 21.5-22.3 3,145 6.5 6.3-6.7 297 0.6 0.5-0.7 1,431 3.0 2.8-3.1 1,245 2.6 2.4-2.7 371 0.8 0.7-0.8 1,456 3.0 2.9-3.2
Female 7,969 17.2 16.8-17.6 2,317 5.0 4.8-5.2 259 0.6 0.5-0.6 766 1.7 1.5-1.8 938 2.0 1.9-2.2 342 0.7 0.7-0.8 1,086 2.3 2.2-2.5
Interrupted aortic arch (IAA)c
 Pulmonary valve atresia
 Single ventricle
 Tetralogy of Fallot (TOF)
 Total anomalous pulmonary venous connection(TAPVC)
 Tricuspid valve atresiad
Variable values Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe Count Prevalence 95% CIe
Total 811 0.9 0.8-0.9 942 1.0 0.9-1.1 852 0.9 0.8-1.0 4,592 4.8 4.7-5.0 1,234 1.3 1.2-1.4 813 0.9 0.8-0.9
Case ascertainment methodology a
Active case finding 331 0.8 0.7-0.9 616 1.4 1.3-1.5 318 0.7 0.6-0.8 2,125 4.8 4.6-5.1 653 1.5 1.4-1.6 432 1.0 0.9-1.1
Passive case finding 480 0.9 0.9-1.0 326 0.6 0.6-0.7 534 1.1 1.0-1.1 2,467 4.9 4.7-5.1 581 1.1 1.1-1.2 381 0.7 0.7-0.8
Maternal race/ethnicity f
White, Non-Hispanic 383 0.8 0.8-0.9 359 0.8 0.7-0.9 359 0.8 0.7-0.9 2,141 4.7 4.5-4.9 442 1.0 0.9-1.1 357 0.8 0.7-0.9
Black, Non-Hispanic 162 1.1 0.9-1.3 184 1.2 1.0-1.4 170 1.1 1.0-1.3 861 5.7 5.3-6.1 178 1.2 1.0-1.4 170 1.1 1.0-1.3
Hispanic 214 0.8 0.7-0.9 304 1.2 1.0-1.3 258 1.0 0.9-1.1 1,180 4.5 4.3-4.8 493 1.9 1.7-2.1 227 0.9 0.8-1.0
Asian or Pacific Islander, Non-Hispanic 22 0.4 0.3-0.6 60 1.1 0.8-1.4 38 0.7 0.5-1.0 259 4.7 4.2-5.3 90 1.6 1.3-2.0 34 0.6 0.4-0.9
American Indian or Alaska Native, Non-Hispanic 5 1.0 0.3-2.3 20 3.8 2.3-5.9 3 0.6 0.1-1.7 41 7.8 5.6-10.6 14 2.7 1.5-4.5 11 2.1 1.0-3.8
Maternal age (years) f
<35 659 0.8 0.8-0.9 771 1.0 0.9-1.1 689 0.9 0.8-0.9 3,514 4.5 4.3-4.6 1,041 1.3 1.2-1.4 651 0.8 0.8-0.9
35+ 145 0.9 0.8-1.1 166 1.0 0.9-1.2 157 1.0 0.8-1.1 1,050 6.5 6.1-6.9 187 1.2 1.0-1.3 157 1.0 0.8-1.1
Infant sex f
Male 437 0.9 0.8-1.0 501 1.0 0.9-1.1 487 1.0 0.9-1.1 2,532 5.2 5.0-5.4 708 1.5 1.4-1.6 430 0.9 0.8-1.0
Female 374 0.8 0.7-0.9 438 0.9 0.9-1.0 364 0.8 0.7-0.9 2,051 4.4 4.2-4.6 525 1.1 1.0-1.2 381 0.8 0.7-0.9
a

Programs with active case-finding methodology: Arizona, California, Delaware, Georgia (Metropolitan Atlanta), Louisiana, North Carolina, Minnesota, Puerto Rico, South Carolina, Texas, Utah (total live births=4,388,631). Programs with passive case-finding methodology: Colorado, Florida, Illinois, Kentucky, New Jersey, New York, Tennessee, Virginia (total live births=5,080,694). Birth defect surveillance programs may have modified the requested code ranges used to define a select defect as necessary. Programs provided the code ranges used to define each birth defect where they differed from those requested by the National Birth Defects Prevention Network (NBDPN) [Table 1]. If a program defined a birth defect using a different code range then the created estimates use the program-specific code range, when no alternate code range was specified the NBDPN code range was used.

b

The CCHDs column displays a count, prevalence, and CI for cases with any of the individual CCHDs presented in the table. Cases with more than one CCHD code were counted only once in this summarized category.

c

Excludes Minnesota in birth year 2014.

d

Select states could not exclude cases with stenosis.

e

CI: Confidence interval calculated using exact Poisson methodology.

f

Counts across subgroups may not add to the total due to other/unknown categories (not shown).