Congenital complete atrioventricular block in children: pathogenesis and clinical outcomes
Udink ten Cate, F.E.A.; Breur, J.M.P.J.; van Woerkom, J.M.; Kruize, A.A.; Stoutenbeek, P.; Meijboom, E.J.
Nederlands Tijdschrift Voor Geneeskunde 146(38): 1777-1781
2002
ISSN/ISBN: 0028-2162 PMID: 12369438 Document Number: 546272
Congenital complete atrioventricular block (CCAVB) is induced by the placental transmission of maternal anti-SS-A/Ro and anti-SS-B/La antibodies during the second trimester of pregnancy where they cause inflammatory injury to the foetal heart. Anti-SS-A/Ro and anti-SS-B/La antibodies can be detected in most mothers of children with CCAVB. However, the chance of an antibody-positive woman giving birth to a child with CCAVB is 1-5% and the chance of this recurring is 16%. In addition to the maternal antibodies, foetal and environmental factors may also play a role in the pathogenesis. CCAVB is associated with high morbidity and mortality during the foetal and neonatal period. Pacemaker implantation is indicated in approximately 60% of these children, after the development of symptoms related to bradycardia, although the timing of this is controversial. The effectiveness of therapeutic intervention in the uterus has yet to be determined. A conservative approach is advisable with respect to the use of corticosteroids.