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Başkent Üniversitesi Tıp Fakültesi ¹Pediatri Uzmanı, ²Pediatri Doçenti 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. Anahtar Kelimeler : Neonatal lupus sendromu, konjenital kalp bloğu, anti La/SSBanti Ro/SSA antikorları, kalp pili.