Circulation, Vol 56, 82-90, Copyright © 1977 by American Heart Association
CM McCue, ME Mantakas, JB Tingelstad and S Ruddy
Of 22 children with congenital complete heart block (CCHB) available for
study, 14 (63.6%) were born to 11 mothers with clinical or laboratory
evidence of connective tissue disease, primarily lupus erythematosus (LE).
Seven mothers had both clinical and laboratory evidence of disease while
four had only positive laboratory studies including fluorescent antinuclear
antibody, rheumatoid factor, and depressed complement levels. In adults
with systemic LE, pathologic changes in the collagen surrounding the
conduction system have led to the fibrosis and death from heart block.
Antinuclear antibodies of the IgG class cross the placental barrier and
newborn infants have been reported with transient skin lesions of lupus.
Placental transmission of such antibodies may affect the fetal cardiac
conduction system, surrounding collagen, and myocardium, leading in some
cases to CCHB. This is probably one important etiologic factor in CCHB even
though the mother is asymptomatic during her pregnancy.
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