TABLE V.
Comparison of Laterality Defects Prevalence (per 10,000) in the Literature and the National Birth Defects Prevention Study, 1998–2007
| Author Year | Study (years) | Total births Total CHD cases Years of study |
Method/Population | Definitions (verbatim from article) | Total No. cases prevalence | Heterotaxy No. cases prevalence | SI, Totalis No. cases prevalence |
|---|---|---|---|---|---|---|---|
| Fyler et al., 1986 | New England Regional Infant Cardiac Program (1968–1974) | 1,083,083 2,381 6 years |
Cardiology centers New England Approximates population-based |
Heterotaxia (Dextro-, meso-, levo-, asplenia); included ectopia cordis | 95 0.9 |
NS | NS |
| Ferencz et al., 1997 | Baltimore-Washington Infant Study (1981–1989) | 906,626 4,390 8 years |
Population-based Metropolitan Baltimore-Washington, D.C. |
Laterality and cardiac looping defects (excluding ectopiacordis) | 104 1.4 |
0.50 | 0.1 |
| Lin et al., 2000 | Brigham and Women’s Hospital (1972–3/99, except 1975–78 | 201,084 489 nontransfers 24 years |
Hospital-based Boston, MA Includes TOP |
Heterotaxy Syndromes included |
NS | 58 total 20 nontransfers 0.99 |
NS |
| Reller et al., 2008 | Metropolitan Atlanta Congenital Defects Program (1998–2005) | 398,140 3,240 6 years |
Population-based Plus local cardiology center. Metropolitan Atlanta, GA |
“Heterotaxy Syndromes” broadly defined | 68 1.7 |
NS | NS |
| Bedard et al., 2012 | Alberta Congenital Anomalies Surveillance System (1995–2002) | 301,899 3,751 8 years |
Population-based Passive registry, Plus two cardiology centers Western Canada Includes TOP |
Heterotaxy NBDPS classification Not subdivided into situsinversus and heterotaxy. Syndromes included |
39 1.3 |
NS | NS |
| Khoshnood et al., 2013 | EPIdemiologie des CARDiopathiescongenitales (EPICARD) (2005–2008) | 317,538 births 2,867 cases 3 years |
Population-based Greater Paris Includes TOP |
Heterotaxy Including isomerism and “mirror-imagery”. Exclusion of chromosome or “other” anomalies applied to total CHD analysis, not specific CHDs |
37 1.2 |
NS | NS |
| Lin et al., 2014 (This study) | NBDPS (1998–2007) All sites |
4,664,529 12,445 10 years |
Population-based 10 sites National USA 5 sites with TOP |
Situsinversustotalis and heterotaxy. Syndromes excluded. |
517 1.11 |
378 0.81 |
139 0.30 |
CHD, congenital heart defects; National Birth Defects Prevention Study (NBDPS); NS, not stated; TOP, termination of pregnancy.