Congenital complete atrioventricular block (CCAVB) is a rare condition of considerable interest and importance to a number of different subspecialty medical and paramedical professional groups, including Pediatric and Adult Cardiologists and Cardiac Surgeons; Pacing Physicians and Technologists; Pediatricians, Obstetricians, Perinatologists, Midwives and Fetal Ultrasonographers; and Rheumatologists, Immu-nologists and Pathologists. Estimates of incidence have varied from 1:15,000 to 1:25,000 live births. It is now recognised that CCAVB represents a major part of the Neonatal Lupus Erythematosus Syndrome, which consists of subacute cutaneous Lupus (which is usually transient and resolves before 6 months of age), complete heart block, and rarely transient hepatic and hematologic manifestations.
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