Abstract
INTRODUCTION: Neonatal lupus syndrome (NLS) is a passively acquired autoimmune condition due to the transplacental passage of maternal anti-Ro/SSA and anti-La/SSB antibodies in mothers with systemic lupus erythematosus (SLE), and congenital complete heart block (CHB) is its most serious manifestation. Skin and hepatic involvement may occur in later infancy. CASE PRESENTATION: A term infant with fetal bradycardia, detected at the 23rd gestational age, was diagnosed with CHB due to NLS and was successfully treated with a permanent epicardial pacemaker. The patient was reported here due to rarity of the procedure in neonatal period. CONCLUSIONS: Mothers with SLE should be screened and closely followed up during pregnancy for the development of fetal atrioventricular (AV) block.