The incidence of CHB is 2 % in cases of maternal anti-Ro/ SSA antibody positivity, 3 % when both anti Ro/SSA and anti-La-SSB are positive. The risk of recurrence is 9 times higher in the subsequent pregnancies [1]. Underlying structural congenital heart disease is associated with more than half of the fetuses found to have CHB and it is then defined ‘‘isolated’’ in the absence of structural hea...

The atrioventricular (AV) node is permanently damaged in approximately 3% of congenital heart surgery operations, requiring implantation of a permanent pacemaker. Improvements in pacemaker design and in alternative treatment modalities require an effective in vivo model of complete heart block (CHB) before testing can be performed in humans. Such a model should enable accurate, reliable, and de...

Background Neonatal Lupus Erythematodus (NLE) is a rare disease occurring in offspring from mothers with anti-Ro with or without anti-La antibodies. Much is known about the individual manifestations (congenital heart block (CHB), cutaneous rash, hematologic and hepatic laboratory abnormalities and macrocephaly) but it is unclear how these and other autoantibodies (anti-dsDNA, anti-RNP and anti-...

This is a pathophysiological correlation in two cases showing split His bundle potentials. The first case had a history of previous complete heart block and the electrophysiological studies revealed split His potentials with intact A-V conduction. Case two had split His potentials with complete heart block. Serial sections of the conduction system in both cases revealed calcific impingement on,...

To correlate the arrhythmogenic effects of maternal autoantibodies with the genesis of congenital heart block, female BALB/c mice were immunized with human recombinant 48-kDa SSB/La, 60-kDa SSA/Ro, 52-kDa SSA/Ro (52alpha), and 52beta (amino acids 169-245 deleted) as well as with murine recombinant 52-kDa SSA/Ro. Control animals received beta-galactosidase or a polypeptide encoded by pET-28 alon...