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Autoantibody-associated congenital heart block (CHB), also know as neonatal lupus, is responsible for the majority (~60-90%) of CHB
This is secondary to maternal antibodies that cross the placenta and may disappear postnatal
Neonatal lupus can result in diffuse myocardial disease both with and without conduction disturbances, structural defects, and electrophysiologic anomalies
Overall mortality is up to 30%, with 15% mortality before 3 months of age
More than 65% of surviving newborns require pacemakers
Maternal screening and fetal echocardiography has allowed routine prenatal diagnosis
References
Capone C, Buyon J, Friedman D, Frishman W. Cardiac Manifestations of Neonatal Lupus: A Review of Autoantibody-associated Congenital Heart Block and its Impact in an Adult Population. Cardiology Review. 2012, Mar-Apr;20(2):72-76