BACKGROUND Congenital complete heart block is a life-threatening condition which is highly associated with autoimmune and connective tissue disorders. Presence of maternal autoantibodies for associated conditions increases the risk of delivering a child with congenital complete heart block, however, less than a half of all women with such antibodies are symptomatic even after delivery. Mortalit...

Congenital heart block (CHB) is the most severe manifestation of Neonatal lupus erythematosus syndrome, which is often described as a model of passively acquired autoimmunity. CHB constitutes a unique model where specific maternal autoantibodies target and mediate fetal organ-specific disease. The essay will first review the studies that are at the basis of the definition of autoantibody-associ...

Clinical data of a 39-year-old patient with corrected transposition the great arteries, combined complete atrioventricular block and conduction along an accessory pathway, are presented. The features changes in structure system, rhythm characteristic this rare congenital heart disease discussed.

The diagnosis of congenital complete atrioventricular block usually offers little difficulty. There remains, however, the problem of interpreting certain clinical findings, including systolic and diastolic murmurs and cardiomegaly, which are sometimes falsely suggestive of an associated congenital heart lesion. The clinical, radiologie, and electrocardiographic findings in 27 children with cong...

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 ...

Introduction Congenital heart block (CHB) has long been associated with maternal anti-SSA/Ro antibodies. Injury to the conduction system is presumed to be a consequence of the transplacental passage of maternal IgG autoantibodies into the fetal circulation. Within the heart, a spectrum of phenotypes has been described, including structural defects, functional abnormalities, and electrical distu...