Abstract
110 120 Congenital complete heart block (CHB) in the absence of cardiac malformations occurs in approximately 1 in 10,000 live births. It is the most serious fetal complication of maternal autoimmune disease, leading to antibody-mediated injury of the fetal atrioventricular node and resulting in fetal bradycardia and low output. Hydrops develops in up to 42% of these fetuses at 27.6 5.1 weeks gestation and is associated with high mortality (>90%). Although resolution of hydrops can occur with sympathomimetic treatment, or even spontaneously, a permanent pacemaker is required in approximately 66% of cases, with placement immediately after birth. Although fetal pacing has been attempted, there are no reports of survival beyond the intraoperative period.
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