A woman with SLE or other autoimmune disease may give birth to an infant with autoimmune congenital heart block (CHB).
ECG abnormalities may include:
(1) atrioventricular (AV) block, often first degree
(2) QTc interval prolongation
Autoimmune diseases associated with congenital heart block:
(1) systemic lupus erythematosus (SLE), with child having the neonatal lupus syndrome (NLS)
(2) Sjogren's syndrome
(3) undifferentiated connective tissue disease
Clinical and laboratory features:
(1) The heart block is due to autoantibodies against calreticulin (CALR, a major calcium binding protein).
(2) The mother is often positive for anti-Ro (SSA) and/or anti-La (SSB).
