Autoimmune-mediated congenital heart block

Autoimmune-mediated congenital heart block (CHB) is a severe manifestation of neonatal lupus in which conduction tissues of the fetal heart are damaged. This occurs due to passive transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies and subsequent inflammation and fibrosis of the atri...

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Bibliographic Details
Published in:Best practice & research. Clinical obstetrics & gynaecology 2020-04, Vol.64, p.41-51
Main Authors: Wainwright, Benjamin, Bhan, Rohit, Trad, Catherine, Cohen, Rebecca, Saxena, Amit, Buyon, Jill, Izmirly, Peter
Format: Article
Language:English
Subjects:
Anti-SSA/Ro autoantibodies
Congenital heart block
Neonatal lupus
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Summary:Autoimmune-mediated congenital heart block (CHB) is a severe manifestation of neonatal lupus in which conduction tissues of the fetal heart are damaged. This occurs due to passive transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies and subsequent inflammation and fibrosis of the atrioventricular (AV) node. Notably, the disease manifests after the fetal heart has structurally developed, ruling out other anatomical abnormalities that could otherwise contribute to the block of conduction. Complete AV block is irreversible and the most common manifestation of CHB, although other cardiac complications such as endocardial fibroelastosis (EFE), dilated cardiomyopathy, and valvular insufficiency have been observed. In this review, we detail the classification, prevalence, pathogenesis, and clinical management recommendations for autoimmune CHB.
ISSN:1521-6934
1532-1932
DOI:10.1016/j.bpobgyn.2019.09.001