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Immune-mediated congenital heart block (CHB): identifying and counseling patients at risk for having children with CHB.

Abstract：

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.

journal_name

Semin Arthritis Rheum

journal_title

Seminars in arthritis and rheumatism

authors

Julkunen H,Kaaja R,Siren MK,Mack C,McCready S,Holthöfer H,Kurki P,Maddison P

doi

10.1016/s0049-0172(98)80042-5

subject

Has Abstract

pub_date

1998-10-01 00:00:00

pages

97-106

issue

eissn

0049-0172

issn

1532-866X

pii

S0049-0172(98)80042-5

journal_volume

pub_type

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Immune-mediated congenital heart block (CHB): identifying and counseling patients at risk for having children with CHB.