Congenital heart block is a rare disorder. It has an incidence of about 1 in 22,000 live
births. It may be associated with high mortality and morbidity. This should generate a
high index of suspicion for early diagnosis and aggressive therapy when appropriate.
The congenital heart block associated with neonatal lupus is considered a form of
passively acquired autoimmune disease in which maternal autoantibodies to the
intracellular ribonucleoproteins Ro (SS-A) and La (SS-B), cross the placenta and
injure the previously normal fetal heart. Women with serum titers of anti-Ro antibody
carry a 3% risk of having a child with neonatal lupus syndrome. Recurrence rates are
about 18%. We believe that serial echocardiograms should be acquired so that early
diagnosis is made and aggressive therapy administered, if signs of conduction
system disease such as PR interval prolongation by Doppler are found, so as to
optimize the outcome. Establishment of guidelines for therapy have been set
empirically, should signs of congenital heart block develop. Those patients whose
congenital heart block is associated with structural heart disease have a higher
morbidity and mortality, which is determined more by the underlying structural
congenital heart disease than it is by the need for a pacemaker per se.peer-reviewe