This case demonstrates that congenital complete heart block associated with neonatal lupus erythematosus can present atypically late and show postnatal improvement in conduction, though clinical deterioration may still occur due to cardiomyopathy.
Alerts clinicians to cardiomyopathy risk despite conduction gains in neonatal lupus; leaves open optimal delivery/IVIG timing in atypical cases.
Complete congenital heart block (CCHB) accompanied with neonatal lupus erythematosus is caused by an immune reaction between maternal anti-Ro/SSA antibodies and the fetal heart and is generally considered an irreversible process. This reaction mostly occurs before 30 weeks' gestation, especially between the 20th and 24th week. Reported here is an atypical case of neonatal lupus erythematosus with CCHB detected after 32 weeks' gestation that showed postnatal improvement in the degree of heart block after preterm delivery and immunoglobulin use. The clinical condition of the infant worsened with an increase in heart rate due to possible cardiomyopathy induced by the immune reaction.
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Isayama et al. (2013) studied this question.
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