INCREASED RECURRENCE RATE OF CONGENITAL HEART BLOCK IN FEMAEE OFFSPRINGS

Journal/Book: Z Rheumatol 1999; 58 Suppl. 1: I/45 (P 28). 1999;

Abstract: Dept. Medicine III and 1Dept. Gynecology and Obstetrics Charite Humboldt University Deutsches Rheumaforschungszentrum Berlin Congenital heart block (CHB) is typically found to be associatod with maternal autoantibodies against the autoantigens 52 kD Ro(SS-A) and La(SS-B) although the majority of mothers with CHB children are healthy. While the coincidence of persistent maternal autoantibodies and CHB are thought to be of pathogenic importance however the relative recurrence rate for CHB has been calculated in the range of 8 % - 18 % indicating the influence of additional factors. Therefore an analysis assessed whether gender is related to the susceptibility to acquire CHB in a cohort of 25 mothers with altogether 27 CHB pregnancies (group I). Most notably an overall predominance of affected girls with CHB (n = 19) in contrast to boys with CHB (n = 8) gave evidence that a genetic and gender related predisposition enhances remarkably CHB susceptibility for girls. To further characterize the influence of gender for the development of CHB the female/male ratio of two additional cohorts were compared with those of our CHB group. First a previously characterized group of newborus with a variety of non-autoimmune cardiac conduction abnormalities (14 female/18 male) without maternal autoantibodies (group II) and secondly a group of hcalthy offsprings who were born by SLE patients (group III; 22 female/23 male). Whereas there was no difference in the female/male ratio between these two control groups as well as between the group of CHB children and the healthy offsprings of SLE patients (group III) comparison of the CHB group with the children suffering from cardiac conduction defects without autoimmune findings (group II) revealed a significant preference for girls to acquire an CHB (Odds ratio 3.054; 95 % confidence interval p < 0.04). ... le

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