Congenital complete atrioventricular block (CCAVB) is an uncommon but potentially serious cardiac condition.
Conditions associated with CCAVB:
(1) structural cardiac malformations
(2) maternal autoimmune disease (usually SLE) with autoantibodies to SS-A/Ro and/or SS-B/La ribonucleoproteins
(3) genetic mutation in the SCN5A or NKX2.5 pathways (which may have a familial pattern of inheritance)
Structural cardiac malformations associated with CCAVB include:
(1) anomalies of great vessel development (correct transposition, etc)
(2) univentricular heart
Cardiac pacing can be used to restore cardiac function in infected infants.
