ISOLATED CONGENITAL COMPLETE HEART-BLOCK - LONG-TERM OUTCOME OF MOTHERS, MATERNAL ANTIBODY SPECIFICITY AND IMMUNOGENETIC BACKGROUND

Objective, To assess the longterm outcome of mothers of children with isolated congenital complete heart block (CCHB), and the maternal spec ific immunoblot pattern and HLA antigens. Methods, Fifteen mothers of 16 children with isolated CCHB were investigated; their followup exten ded up to 15.8 years on average after the index delivery. Anti-Re and La antibodies were detected by counterimmunoelectrophoresis and ELISA; anti-Re antibodies were studied by immunoblot. HLA typing was done us ing a microcytotoxicity test. Results, One mother has systemic lupus e rythematosus (SLE) before the index delivery. The other mothers develo ped only minor symptoms (arthralgia, dry eyes and photosensitivity) re sembling primary Sjogren's syndrome more than classic lupus. All 15 mo thers were anti-Re and 9 were also anti-La positive, a mean of 12.5 ye ars after the index delivery. Eight mothers reacted with the 52 kDa SS A(Ro) component, and 2 also with the 60 kDa SSA(Ro) component. The pre valence of the DR3 antigen and of the B44/DR5, DR3/DQ2 and Al/Cw7/B8/D R3/DQ2 haplotypes was significantly increased. Conclusion, The longter m outcome for the mothers of children with CCHB is more reassuring tha n generally assumed. All the mothers were anti-Re positive by sensitiv e ELISA. Reactivity to the denaturated 52 kDa SSA(Ro) component seems characteristic of these mothers, who presented a particular immunogene tic background.