Congenital heart block – neonatal lupus syndrome

Congenital atrioventricular block, diagnosed in utero, at birth, or within the neonatal period, is a rare disorder closely linked to transplacental transport of maternal autoantibodies anti-Ro/SSA and anti-La/SSB. Complete atrioventricular block is the most serious and irreversibile clinical manifestation of the neonatal lupus syndrome. In this article we describe three patients with complete congenital atrioventricular block who were observed in our hospital during the last 8 years. Neither of these cases was caused by structural heart defects, and the mothers were recognized as having an autoimmune connective tissue disease only after the delivery. Children are developing normally. There are no cardiac symptoms that may be associated with the complete heart block. On the bases of their ventricular rates there were no indications for pacing. None of the patients have skin, liver or hematologic lesions. Complete atrioventricular block is the only clinical manifestation in these cases.