The clinical spectrum of autoimmune congenital heart block

Pathophysiology of autoimmune-associated congenital heart block

Congenital heart block (CHB), detected at or before birth, in a structurally normal heart, is strongly associated with autoantibodies to SSA/Ro-SSB/La ribonucleoproteins. The historical hallmark of CHB is complete atrioventricular block (AVB) which is irreversible. CHB occurs in 2% of primigravid mothers with anti-Ro/La antibodies, and in 20% of women who had a previous CHB offspring. Although ...

Which Intrauterine Treatment for Autoimmune Congenital Heart Block?

Autoimmune Congenital Heart Block (CHB) is considered an immune mediated manifestation, caused by the action of maternal autoantibodies anti-Ro/SSA and anti-La/SSB on fetal cardiac tissues. The incidence of CHB is 2% in anti-Ro/SSA positive women, 3% when both anti-Ro/SSA and anti-La-SSB are positive. In the subsequent pregnancies the risk of recurrence is 9 times higher. The antenatal diagnosi...

Neonatal congenital heart block.

Congenital Heart Block (CHB) is the most serious complication of neonatal lupus erythematosus. Transplasental transfer of maternal anti SSA/Ro or antiSSB/La antibodies around 12th week of gestation is associated with development of CHB. This may lead to inflammation, fibrosis and scarring of fetal conduction system in the early second trimester. Different degrees of atrioventricular (AV) block ...

Congenital complete heart block.

Congenital partial heart block.

Although congenital complete heart block is well recognized and has been fully reviewed, congenital partial heart block appears to be much more unusual. Howevev, a survey shows that several such cases have been described in America, but we have found only four cases recorded from this country. Whipham (1915) reported two, and Fleming and Stevenson (1928) gave an account of an eight-year-old gir...