Approximately one percent of pregnant women will produce anti-Sjogren's syndrome-related antigen A (anti-Ro/SSA) antibodies. Of these pregnancies, one to three percent will have a fetus that develops atrioventricular (AV) block. Earlier stages of AV block (1° or 2°) may respond to anti-inflammatory treatment, but complete (3°) AV block, which can occur within 24 hours of a normal fetal rhythm, is likely irreversible and carries substantial risk for significant morbidity and for mortality. Emerging data has shown that ambulatory fetal heart rhythm monitoring can detect the transition period from normal rhythm to 2° AV block, the time during which treatment with IVIG and dexamethasone can potentially restore normal sinus rhythm. Weekly or biweekly fetal echocardiograms occur too infrequently to detect this transition period but may still be useful in diagnosing extranodal anti-Ro antibody mediated cardiac disease. In this review, we evaluate the most innovative methods for surveillance and treatment of this disease.
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