1. Autoimmune-mediated congenital heart block
- Author
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Catherine Trad, Rebecca E. Cohen, Peter M. Izmirly, Rohit Bhan, Amit Saxena, Jill P. Buyon, and Benjamin J. Wainwright
- Subjects
Adult ,medicine.medical_specialty ,Inflammation ,Disease ,030204 cardiovascular system & hematology ,Autoantigens ,Congenital heart block ,Autoimmune Diseases ,Pathogenesis ,03 medical and health sciences ,Fetal Heart ,0302 clinical medicine ,Pregnancy ,Fibrosis ,Internal medicine ,medicine ,Humans ,Autoantibodies ,030203 arthritis & rheumatology ,Lupus Vulgaris ,business.industry ,Autoantibody ,Obstetrics and Gynecology ,Dilated cardiomyopathy ,Endocardial fibroelastosis ,General Medicine ,medicine.disease ,Pregnancy Complications ,Heart Block ,Cardiology ,Female ,medicine.symptom ,business - Abstract
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.
- Published
- 2020
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