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[Congenital complete atrioventricular block in children: pathogenesis and clinical outcomes]
F E A Udink ten Cate1, J M P J Breur, J M van Woerkom
1Universitair Medisch Centrum Utrecht, Postbus 85.090, 3508 AB Utrecht.
Insights
Maternal antibodies like anti-SS-A/Ro and anti-SS-B/La can cause congenital complete atrioventricular block (CCAVB) in fetuses. While not all antibody-positive mothers have affected children, CCAVB leads to significant fetal and neonatal risks.
Area of Science:
- Cardiology
- Immunology
- Maternal-Fetal Medicine
Context:
- Congenital complete atrioventricular block (CCAVB) arises from placental transfer of maternal anti-SS-A/Ro and anti-SS-B/La antibodies.
- These antibodies target the fetal heart during the second trimester, causing inflammatory injury.
- While most mothers of affected infants have these antibodies, the transmission risk is low (1-5%).
Purpose:
- To review the pathogenesis, clinical implications, and management of congenital complete atrioventricular block.
- To highlight the role of maternal autoantibodies in fetal cardiac development.
- To discuss the associated risks and current treatment controversies.
Summary:
- CCAVB is an inflammatory fetal heart condition caused by maternal autoantibodies.
- Affected infants face high risks of fetal and neonatal morbidity and mortality.
- Pacemaker implantation is required in about 60% of symptomatic cases, with controversial timing.
Impact:
- Informs clinical practice regarding the management of pregnancies at risk for CCAVB.
- Highlights the need for further research into preventative and therapeutic strategies.
- Emphasizes the importance of understanding autoimmune mechanisms in congenital heart defects.
Abstract:
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.