This case series postulates a causative relationship between maternal systemic lupus erythematosus and congenital complete heart block in infants, highlighting associated cardiomyopathy and structural abnormalities.
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Does not support practice changes; hypothesis-generating for pathogenesis of congenital heart block in maternal SLE.
Infants born to mothers with disseminated lupus erythematosus occasionally have transient manifestations of the maternal disease. In six infants with congenital heart block born to mothers with systemic lupus erythematosus we postulated a causative relation. In one of the infants a post-mortem study of the conduction system suggested faulty embryonic development of the atrioventricular node with an abnormally thick annulus fibrosus and the effects of early inflammatory changes. Two of the infants had a cardiomyopathy and three, associated congenital heart disease.
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Chameides et al. (1977) studied this question.
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