Cytogenomic Evaluation of Subjects with Syndromic and Nonsyndromic Conotruncal Heart Defects.

Despite considerable advances in the detection of genomic abnormalities in congenital heart disease (CHD), the etiology of CHD remains largely unknown. CHD is the most common birth defect and is a major cause of infant morbidity and mortality, and conotruncal defects constitute 20% of all CHD cases....

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Published in:BioMed Research International Vol. 2015; pp. 1 - 13
Main Authors: de Souza, Karen Regina, Mergener, Rafaella, Huber, Janaina, Campos Pellanda, Lucia, Riegel, Mariluce
Format: pictorial research tables/charts Journal Article
Published: Wiley-Blackwell 6/7/2015
Online Access:View this record in EBSCOhost
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      dt: 6/7/2015
      vid: 2015
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      pub: Wiley-Blackwell
      place: Malden, Massachusetts
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        109274496
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        10.1155/2015/401941
        109274496
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        atl: Cytogenomic Evaluation of Subjects with Syndromic and Nonsyndromic Conotruncal Heart Defects.
      aug:
        au:
          de Souza, Karen Regina
          Mergener, Rafaella
          Huber, Janaina
          Campos Pellanda, Lucia
          Riegel, Mariluce
        affil: Postgraduate Program in Genetics and Molecular Biology, Universidade Federal do Rio Grande do Sul (UFRGS), 91501-970 Porto Alegre, RS, Brazil
      sug:
        subj:
          Heart Defects, Congenital Familial and Genetic
          Heart Defects, Congenital Etiology
          Retrospective Design
          DNA Blood
          Genomics Methods
          Oligonucleotide Array Sequence Analysis
          Human
          Female
          Male
          Child, Preschool
          Child
          Adolescence
          Adult
          Tetralogy of Fallot
          Transposition of Great Arteries
          Truncus Arteriosus, Persistent
          Descriptive Statistics
          Data Analysis Software
          P-Value
          Blood Donors Brazil
          Brazil
          Funding Source
          Child, Preschool: 2-5 years
          Child: 6-12 years
          Adolescent: 13-18 years
          Adult: 19-44 years
          Female
          Male
      ab: Despite considerable advances in the detection of genomic abnormalities in congenital heart disease (CHD), the etiology of CHD remains largely unknown. CHD is the most common birth defect and is a major cause of infant morbidity and mortality, and conotruncal defects constitute 20% of all CHD cases. We used array comparative genomic hybridization (array-CGH) to retrospectively study 60 subjects with conotruncal defects and identify genomic imbalances. The DNA copy number variations (CNVs) detected were matched with data from genomic databases, and their clinical significance was evaluated. We found that 38.3% (23/60) of CHD cases possessed genomic imbalances. In 8.3% (5/60) of these cases, the imbalances were causal or potentially causal CNVs; in 8.3% (5/60), unclassified CNVs were identified; and in 21.6% (13/60), common variants were detected. Although the interpretation of the results must be refined and there is not yet a consensus regarding the types of CHD cases in which array-CGH should be used as a first-line test, the identification of these CNVs can assist in the evaluation and management of CHD. The results of such studies emphasize the growing importance of the use of genome-wide assays in subjects with CHD to increase the number of genomic data sets associated with this condition.
      pubtype: Academic Journal
      doctype:
        pictorial
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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