Abstract: The autoantibody-related congenital heart block (ACHB) is an autoimmune condition associated with transplacental maternal autoantibodies (anti-SSA/Ro antibodies and anti-SSB/La antibodies) which interfere in the developing heart conduction of the fetus, mostly manifesting as third degree atrioventricular block. The incidence of primary pregnancy in those patients with the positive anti-SSA/Ro antibody is 2%, and 12%-20% in secondary pregnancy. Atrioventricular conduction is the basis of cardiac electric activity and mechanical activity. Atrioventricular block is a disturbance in conduction of sinus or atrial impulse through the specialized conducting system and the severity depends on how fast the ventricular rate is. The incidence of ACHB is low, however, its poor prognosis should be concerned. This review was aimed to provide clinical evidence for screening, treatment and prognosis of this disease by describing the pathogenesis, risk factors, diagnostic methods, prognosis and prenatal interventions.

Key words: Heart block, Atrioventricular block, Autoimmune diseases, Fetus, Anti-SSA/Ro antibodies, Anti-SSB/La antibodies