Molecular mechanisms of congenital heart block
- PMID: 24434353
- DOI: 10.1016/j.yexcr.2014.01.003
Molecular mechanisms of congenital heart block
Abstract
Autoantibody-associated congenital heart block (CHB) is a passively acquired autoimmune condition associated with maternal anti-Ro/SSA antibodies and primarily affecting electric signal conduction at the atrioventricular node in the fetal heart. CHB occurs in 1-2% of anti-Ro/SSA antibody-positive pregancies and has a recurrence rate of 12-20% in a subsequent pregnancy. Despite the long-recognized association between maternal anti-Ro/SSA autoantibodies and CHB, the molecular mechanisms underlying CHB pathogenesis are not fully understood, but several targets for the maternal autoantibodies in the fetal heart have been suggested. Recent studies also indicate that fetal susceptibility genes determine whether an autoantibody-exposed fetus will develop CHB or not, and begin to identify such genes. In this article, we review the different lines of investigation undertaken to elucidate the molecular pathways involved in CHB development and reflect on the hypotheses put forward to explain CHB pathogenesis as well as on the questions left unanswered and that should guide future studies.
Keywords: Atrioventricular block; Autoantibodies; Congenital heart block; La/SSB; MHC; Neonatal lupus erythematosus; Ro/SSA.
Copyright © 2014 Elsevier Inc. All rights reserved.
Similar articles
-
Neonatal lupus: bedside to bench and back.Scand J Rheumatol. 1996;25(5):271-6. doi: 10.3109/03009749609104057. Scand J Rheumatol. 1996. PMID: 8921918 Review.
-
[Congenital heart block associated with maternal anti SSA/SSB antibodies :a report of four cases].Pathol Biol (Paris). 2004 Apr;52(3):138-47. doi: 10.1016/j.patbio.2003.06.002. Pathol Biol (Paris). 2004. PMID: 15063933 Review. French.
-
Significantly increased maternal and fetal IgG autoantibody levels to 52 kD Ro (SS-A) and La(SS-B) in complete congenital heart block.J Autoimmun. 1995 Oct;8(5):675-84. doi: 10.1006/jaut.1995.0050. J Autoimmun. 1995. PMID: 8579723
-
Acquired congenital heart block. Pattern of maternal antibody response to biochemically defined antigens of the SSA/Ro-SSB/La system in neonatal lupus.J Clin Invest. 1989 Aug;84(2):627-34. doi: 10.1172/JCI114208. J Clin Invest. 1989. PMID: 2760204 Free PMC article.
-
Congenital heart block: evidence for a pathogenic role of maternal autoantibodies.Arthritis Res Ther. 2012 Apr 26;14(2):208. doi: 10.1186/ar3787. Arthritis Res Ther. 2012. PMID: 22546326 Free PMC article. Review.
Cited by
-
Molecular Mechanisms of Fetal and Neonatal Lupus: A Narrative Review of an Autoimmune Disease Transferal across the Placenta.Int J Mol Sci. 2024 May 10;25(10):5224. doi: 10.3390/ijms25105224. Int J Mol Sci. 2024. PMID: 38791261 Free PMC article. Review.
-
Late-Onset Dilated Cardiomyopathy in Auto Immune-Mediated Complete Congenital Heart Block: A Case Report.Cureus. 2024 Feb 14;16(2):e54222. doi: 10.7759/cureus.54222. eCollection 2024 Feb. Cureus. 2024. PMID: 38496172 Free PMC article.
-
Autoimmune congenital heart block: a case report and review of the literature related to pathogenesis and pregnancy management.Arthritis Res Ther. 2024 Jan 2;26(1):8. doi: 10.1186/s13075-023-03246-w. Arthritis Res Ther. 2024. PMID: 38167489 Free PMC article. Review.
-
Autoimmune Congenital Complete Heart Block: How Late Can It Occur?AJP Rep. 2023 May 8;13(2):e29-e34. doi: 10.1055/s-0043-1768708. eCollection 2023 Apr. AJP Rep. 2023. PMID: 37168107 Free PMC article. Review.
-
Genetics and epigenetics of primary Sjögren syndrome: implications for future therapies.Nat Rev Rheumatol. 2023 May;19(5):288-306. doi: 10.1038/s41584-023-00932-6. Epub 2023 Mar 13. Nat Rev Rheumatol. 2023. PMID: 36914790 Free PMC article. Review.
Publication types
MeSH terms
Substances
Supplementary concepts
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical
Research Materials
