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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.

Seminars in Arthritis and Rheumatism
|November 7, 1998
PubMed

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%).
Abstract

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