POS0751 COMORBIDITY AND LONG-TERM OUTCOME IN PATIENTS WITH CONGENITAL HEART BLOCK: PRELIMINARY DATA OF THE ITALIAN REGISTRY ON THE IMMUNE-MEDIATED CONGENITAL HEART BLOCK

Background: Congenital heart block (CHB) is due to placental transfer of maternal anti-Ro/SSA autoantibodies the fetus. The prevalence CHB has been estimated as 1-2% in women while recurrence rate 16-19% (1). This condition associated with a high fetal/neonatal mortality and most cases requires pacemaker (PM) pacing. Given rarity CHB, limited data are available regarding long-term follow-up offspring other than cardiovascular complications. Objectives: results Italian Registry autoimmune congenital were recently described (2). A peculiarity this cohort was that mothers had an established diagnosis systemic disease at detection, contrast registries where mostly incidentally detected healthy women. Here we report update, preliminary outcome patients their unaffected siblings health controls born from positive for Ro/SSA. Methods: Data demography, treatment, maternal, neonatal outcome, collected through online electronic datasheet. dedicated questionnaire created aim investigate general health, follow-up, frequency diseases. Results: One-hundred five 99 included 1969 December 2020. utero (97 cases, 92.3%) 8 cases. Third degree occurred 71 (67.6%). Child observed 29 (27.6%) cases: 20 utero, 7 during period 2 childhood. Overall, PM implanted 54 out 85 live births (63.5%). Then, our divided into subgroups: pregnancy before (N=61) after 2010 (N=44) evaluate possible differences among subgroups. Whereas mortality, PM, similar, more frequently last 10 years Ro/SSA asymptomatic carriers group pregnancies (53.6% vs 32.8%, p=0.038). Questionnaires 14 surviving 12 collected. Among 6 males females, median age (range 6-28). All presented third required pacing one implantable ECG recorder. substituted least once 9 patients, oldest patient change it four times. No dilated cardiomyopathy maintain annual follow-up. Two diseases registered idiopathic juvenile arthritis Cogan’s vasculitis, both Sjogren Syndrome. Four neurodevelopmental disorders occurred: three learning disabilities (one each group) case speech disorder sibling group. In addition, stress linked frequent hospitalizations. Conclusion: registry ongoing project aiming collecting all CHB. Moreover, here reported concerning evaluation patients. Our data, even if need be confirmed larger cohort, seems reassuring: no comparing or controls. References: [1]Brito-Zéron et al. Nat Rev Rheumatol 2015;11:301-312. [2]Fredi M Front Cardiovasc Med. 2019 Feb 28;6:11. Disclosure Interests: None declared