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Congenital heart defects and major structural noncardiac anomalies in Alberta, Canada, 1995–2002

Authors

  • R. Brian Lowry,

    Corresponding author
    1. Alberta Congenital Anomalies Surveillance System, Alberta Health and Wellness, Calgary, Alberta, Canada
    2. Departments of Medical Genetics and Pediatrics, University of Calgary, Calgary, Alberta, Canada
    3. Alberta Children's Hospital, Alberta Health Services, Calgary, Alberta, Canada
    4. Alberta Children's Hospital Research Institute
    • Alberta Congenital Anomalies Surveillance System/Clinical Genetics, Alberta Children's Hospital, 2888 Shananappi Tr NW, Calgary, Alberta, T3B 6A8 Canada
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  • Tanya Bedard,

    1. Alberta Congenital Anomalies Surveillance System, Alberta Health and Wellness, Calgary, Alberta, Canada
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  • Barbara Sibbald,

    1. Alberta Congenital Anomalies Surveillance System, Alberta Health and Wellness, Calgary, Alberta, Canada
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  • Joyce R. Harder,

    1. Alberta Children's Hospital, Alberta Health Services, Calgary, Alberta, Canada
    2. Division of Pediatric Cardiology, Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada
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  • Cynthia Trevenen,

    1. Alberta Children's Hospital, Alberta Health Services, Calgary, Alberta, Canada
    2. Department of Pathology and Laboratory Medicine, University of Calgary, Calgary, Alberta, Canada
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  • Vera Horobec,

    1. Western Canadian Children's Heart Network, Edmonton, Alberta, Canada
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  • John D. Dyck

    1. Western Canadian Children's Heart Network, Edmonton, Alberta, Canada
    2. Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
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Abstract

BACKGROUND

Although the majority of congenital heart defects (CHDs) occur in isolation, a significant number occur with noncardiac anomalies. This study determined the proportion of noncardiac anomalies among CHD cases in Alberta.

METHODS

Records of infants and children born in Alberta between January 1, 1995, to December 31, 2002, were searched using multiple sources of ascertainment in addition to the Alberta Congenital Anomalies Surveillance System (ACASS) (Alberta Health and Wellness, 2012). Each case was assigned to one CHD category and was further categorized into one of the following groups: isolated CHD, syndromes, chromosomal, associations and sequences, teratogens, Mendelian, neoplasia, heterotaxy, multiple minor anomalies, and multiple major anomalies.

RESULTS

Of all 3751 CHD cases (prevalence 12.42/1000 total births: confidence interval, 12.03–12.83), 75% were isolated, 10% had a chromosomal etiology, and 9% had multiple major anomalies. All other categories accounted for <2% each. The most commonly associated major noncardiac anomalies were musculoskeletal (MSK) (24%) followed by anomalies of the urinary tract (14%), gastrointestinal system (GI) (11%), and central nervous system (CNS) (11%).

CONCLUSIONS

This is both a population-based and clinical study using a classification scheme that could help to determine possible etiologic factors contributing to CHD. By eliminating known etiologies such as chromosomal and single gene, future studies can focus on the remainder to evaluate possible preventive measures. The most commonly associated major noncardiac anomalies involve the MSK system, followed by the urinary, GI, and CNS systems. Birth Defects Research (Part A) 2013. © 2013 Wiley Periodicals, Inc.

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