Congenital heart block in newborns of mothers with connective tissue disease

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Implantation of Total Artificial Heart in Congenital Heart Disease
07:27

Implantation of Total Artificial Heart in Congenital Heart Disease

Published on: July 18, 2014

C M McCue, M E Mantakas, J B Tingelstad

    Circulation
    |July 1, 1977
    PubMed

    Insights

    Maternal lupus erythematosus (LE) and associated antibodies are linked to congenital complete heart block (CCHB) in infants. This placental antibody transmission may cause fetal cardiac conduction system issues, even if the mother shows no symptoms.

    Area of Science:

    • Immunology
    • Cardiology
    • Pediatrics

    Background:

    • Congenital complete heart block (CCHB) is a serious condition affecting newborns.
    • Connective tissue diseases, particularly lupus erythematosus (LE), are implicated in CCHB.
    • Maternal autoantibodies, such as antinuclear antibodies (ANAs), can cross the placenta.

    Purpose of the Study:

    • To investigate the association between maternal connective tissue disease, especially lupus erythematosus, and CCHB in children.
    • To explore the role of transplacental antibody transmission in the etiology of CCHB.

    Main Methods:

    • Retrospective study of 22 children diagnosed with CCHB.
    • Review of maternal medical history and laboratory findings for evidence of connective tissue disease.
    • Analysis of antibody presence (ANAs, rheumatoid factor) and complement levels in mothers.

    Main Results:

    • 14 out of 22 children (63.6%) with CCHB were born to mothers with clinical or laboratory evidence of connective tissue disease, primarily LE.
    • Seven mothers had both clinical and laboratory evidence of LE; four had only positive laboratory markers.
    • Maternal autoantibodies, including IgG ANAs, were identified as potential culprits.

    Conclusions:

    • Maternal lupus erythematosus and associated autoantibodies are a significant risk factor for CCHB in offspring.
    • Placental transfer of maternal IgG autoantibodies may directly impact the fetal cardiac conduction system, leading to CCHB.
    • CCHB can occur even when the mother is asymptomatic during pregnancy, highlighting the importance of antibody screening.

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