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Anti-Ro (SSA) and anti-La (SSB) antibodies and complete congenital heart block
Y Finkelstein1, Y Adler, L Harel
1Department of Pediatrics C Schneider Children's Medical Center of Israel, Petah Tikvah, Israel.
Insights
Complete congenital heart block (CCHB), a severe neonatal lupus complication, results from maternal antibody transfer. Early detection and intervention are crucial for affected newborns and high-risk pregnancies.
Area of Science:
- Immunology
- Cardiology
- Neonatology
Background:
- Complete congenital heart block (CCHB) is a severe manifestation of neonatal lupus syndrome with high mortality.
- Pathogenesis involves transplacental transfer of maternal anti-Ro (SSA) and anti-La (SSB) antibodies, causing cardiac conduction system damage.
- These antibodies are detected in maternal sera and fetal cardiac tissues.
Purpose of the Study:
- To summarize the understanding of CCHB pathogenesis, diagnosis, and management.
- To highlight the role of anti-Ro (SSA) and anti-La (SSB) antibodies in CCHB development.
- To inform clinical practice regarding monitoring and treatment of CCHB.
Main Methods:
- Review of existing literature on CCHB, neonatal lupus, and associated autoantibodies.
- Analysis of pathogenic mechanisms involving maternal autoantibodies.
- Summary of diagnostic findings and therapeutic approaches.
Main Results:
- CCHB is linked to maternal anti-Ro (SSA) and anti-La (SSB) antibodies.
- Prenatal diagnosis necessitates careful monitoring and timely delivery.
- Treatment options are debated, including intrauterine plasmapheresis and corticosteroids, with most infants requiring pacemakers.
Conclusions:
- Maternal anti-Ro (SSA) and anti-La (SSB) antibodies are key in CCHB pathogenesis.
- Management requires multidisciplinary care, including fetal monitoring and postnatal intervention.
- Testing for these antibodies in pregnant women with SLE and affected newborns is recommended.
Abstract:
Complete congenital heart block (CCHB) is the most severe manifestation of neonatal lupus syndrome, associated with a mortality rate of up to 31%. It is caused by irreversible damage to the cardiac conduction system due to the transplacental passage of maternal antibodies to the fetus. Anti-Ro (SSA) and anti-La (SSB) antibodies are usually detected in sera of mothers of children with prenatally diagnosed CCHB and in cardiac tissues of affected newborns. Their pathogeneic role in the development of CCHB has been established in several studies. When CCHB is detected during pregnancy, careful monitoring and delivery are needed before heart failure is developed. Treatment strategies are debatable and may include prophylactic therapy for high-risk pregnant women and a combination of intrauterine plasmapheresis with plasma exchange or with corticosteroids. Pacemaker insertion is required in most infants during the first three months of life. Assays for anti-Ro (SSA) and anti-La (SSB) antibodies should be performed on sera of pregnant women with SLE and newborns with CCHB.
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