Congenital Heart Defects and Major Structural Noncardiac Anomalies, Atlanta, Georgia, 1968 to 2005

doi:10.1016/j.jpeds.2010.12.051

The Journal of Pediatrics

Volume 159, Issue 1, July 2011, Pages 70-78.e2

https://doi.org/10.1016/j.jpeds.2010.12.051 Get rights and content

Objective

To identify the proportion of major structural noncardiac anomalies identified with congenital heart defects (CHDs).

Study design

Records of infants with CHDs in the Metropolitan Atlanta Congenital Defects Program who were born during the period 1968 through 2005 were classified as having isolated, syndromic, multiple CHD (ie, having an unrecognized pattern of multiple congenital anomalies or a recognized pattern of multiple congenital anomalies of unknown etiology), or laterality defects. Frequencies of associated noncardiac anomalies were obtained.

Results

We identified 7984 live-born and stillborn infants and fetuses with CHDs. Among them, 5695 (71.3%) had isolated, 1080 (13.5%) had multiple, 1048 (13.1%) had syndromic, and 161 (2.0%) had laterality defects. The percentage of multiple congenital anomalies was highest for case with atrial septal defects (18.5%), cardiac looping defects (17.2%), and conotruncal defects (16.0%), and cases with atrioventricular septal defects represented the highest percentages of those with syndromic CHDs (66.7%).

Conclusions

Including those with syndromes and laterality defects, 28.7% of case infants with CHDs had associated major noncardiac malformations. Thus, infants with CHDs warrant careful examination for the presence of noncardiac anomalies.

Section snippets

Methods

We identified all live-born and stillborn infants and elective terminations of pregnancy (TOPs) with diagnosed CHDs delivered during the period 1968 through 2005 that were ascertained by the Metropolitan Atlanta Congenital Defects Program (MACDP). MACDP was granted authority to conduct birth defect surveillance in the five central counties of metropolitan Atlanta in collaboration with and on behalf of the Georgia Division of Public Health by the Georgia Department of Human Resources and has

Results

A total of 7984 live-born and stillborn infants and fetuses with CHDs meeting the MACDP case definition were included: 169 (2.1%) with cardiac looping defects, 1204 (15.1%) with conotruncal defects, 447 (5.6%) with AVSDs, 1031 (12.9%) with LVOTOs, 694 (8.7%) with RVOTOs, 1020 (12.8%) with ASDs, 3579 (44.8%) with VSDs, 157 (2.0%) with cell growth defects, and 66 (0.8%) with Ebstein anomaly. Because some infants had more than one independent CHD, the total number of individual defects was greater

Discussion

The objective of our study was to assess the proportion and pattern of structural noncardiac anomalies among children with CHDs from data culled from a large population-based surveillance system. Analysis of our case infants showed that 71.3% had isolated CHDs, 13.5% had an undefined pattern of MCAs, 13.1% had specific syndromes, and 2.0% had laterality defects. The proportion with MCAs was well within the range reported by other epidemiologic studies (5.9% to 14.1%). 1, 10, 12 The slight

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: The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention. The authors declare no conflicts of interest.

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Original ArticleCongenital Heart Defects and Major Structural Noncardiac Anomalies, Atlanta, Georgia, 1968 to 2005

Objective

Study design

Results

Conclusions

Section snippets

Methods

Results

Discussion

J Pediatr

Am J Cardiol

Int J Cardiol

Semin Perinatol

J Am Coll Cardiol

J Pediatr

Lancet

Pediatr Clin North Am

J Pediatr Surg

Gastroenterology

Genetic and Environmental Risk Factors of Major Cardiovascular Malformations. The Baltimore-Washington Infant Study 1981-1989

The epidemiology of cardiovascular defects, I: a study based on data from three large registries of congenital malformations

Pediatr Cardiol

Moss and Adam’s Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult

Spectrum of congenital heart defects and extracardiac malformations associated with chromosomal abnormalities: results of a seven year necropsy study

Heart

Malcić I. Spectrum of congenital heart defects in Croatia

Eur J Pediatr

Noncardiac malformations in congenital heart disease: a retrospective analysis of 305 pediatric autopsies

Turk J Pediatr

Malformation patterns in children with congenital heart disease

Am J Dis Child

Original Article
Congenital Heart Defects and Major Structural Noncardiac Anomalies, Atlanta, Georgia, 1968 to 2005