Isolated congenital heart block without intracardiac anatomic malformations is a rare cardiac disorder often detected at or before birth. This condition is frequently associated with neonatal lupus syndrome (NLS), a model of passively acquired autoimmunity due to the transplacental transfer of maternal anti La/SSB and/or anti Ro/SSA antibodies through the fetal circulation. Neonatal lupus accounts for 90-95% of cases of heart block occurring in utero or in the neonatal period. Isolated congenital heart blocks are usually complete and require a pacemaker in about 66% of cases. Here, we report two preterm infants with neonatal lupus syndrome who presented with isolated complete congenital heart block and were successfully treated with pacemaker implantation.

Keywords: Neonatal lupus sendromu, konjenital kalp bloğu, anti La/SSBanti Ro/SSA antikorları, kalp pili.