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Immune-mediated congenital heart block (CHB): identifying and counseling patients at risk for having children with
H Julkunen1, R Kaaja, M K Siren
1Peijas Hospital, Helsinki University Hospital, Finnish Red Cross Blood Transfusion Service, and University of Helsinki.
Insights
Maternal antibodies like anti-SS-A/Ro and anti-SS-B/La indicate risk for congenital heart block (CHB) in infants. While no single antibody is unique, testing can identify mothers at high or low risk for CHB.
Area of Science:
- Immunology
- Obstetrics
- Pediatrics
Background:
- Congenital heart block (CHB) is a serious condition affecting newborns.
- Maternal autoantibodies, particularly against SS-A/Ro and SS-B/La antigens, are implicated in CHB pathogenesis.
- Identifying at-risk mothers is crucial for counseling and potential intervention.
Purpose of the Study:
- To determine maternal antibody patterns associated with an increased risk of CHB.
- To establish a basis for counseling women with a history of CHB-affected children.
Main Methods:
- Retrospective clinical study of obstetric histories from 46 Finnish women with CHB-affected children.
- Comparison of immune responses to SS-A/Ro and SS-B/La using immunoblot and ELISA.
- Inclusion of control groups: 85 women with systemic lupus erythematosus (SLE) and 32 with primary Sjögren's syndrome (SS) and healthy children.
Main Results:
- High levels of anti-SS-A/Ro and anti-SS-B/La antibodies significantly increased CHB risk.
- Anti-52 kd SS-A/Ro by immunoblot was the best single predictor (OR 18.9).
- Low or undetectable antibody levels indicated low CHB risk. Female infants had a higher risk (1.9x).
Conclusions:
- Maternal antibody profiles can identify high and low CHB risk, though no single profile is unique.
- Female infants have an increased risk of CHB.
- The risk of a mother having another CHB-affected child is relatively low (12%).
Objective:
To identify patterns of maternal antibodies associated with an increased risk of having a child with congenital heart block (CHB) and to provide a basis for counseling women with a previously affected child.
Methods:
This retrospective clinical study of the obstetric histories of 46 Finnish women with a CHB child compared the strength and specificity of the immune response to SS-A/Ro and SS-B/La, as determined by immunoblot and ELISA, in 44 affected women with 85 women with systemic lupus erythematosus (SLE) and 32 women with primary Sjögren's syndrome (SS) with healthy children.
Results:
High levels of anti-SS-A/Ro and anti-SS-B/La by practically all assays were associated with a significantly increased risk of having a CHB child. The best single test to identify high-risk mothers was anti-52 kd SS-A/Ro by immunoblot (OR 18.9), and it was the only assay to detect mothers at increased risk of CHB as compared with controls with primary SS. Low risk of CHB was indicated by undetectable or low levels of antibodies in the ELISA assays and no reactivity on immunoblot. Mothers with a previous child with CHB had a history of fetal loss (mostly spontaneous abortions) or a history of recurrent fetal losses (> or = 3) slightly more often than controls. Late-trimester obstetric complications in non-CHB pregnancies were insignificant. The relative risk for a female child compared with a male child to have CHB was 1.9 (1.2-2.9, P = .009), and the risk of the mother having another child with CHB was 12% (4 of 34).
Conclusion:
Although there is no unique antibody profile specific for CHB, mothers with a high or low risk of having a child with CHB can be identified. Female children appear to have an increased risk of CHB, but the risk of the mother having another child with CHB is low.