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CC BY-NC-ND 4.0 · AJP Rep 2023; 13(02): e29-e34
DOI: 10.1055/s-0043-1768708
Review Article

Autoimmune Congenital Complete Heart Block: How Late Can It Occur?

Luv Makadia 
1   Division of Pediatric Cardiology, Department of Pediatrics, Hassenfeld Children's Hospital at NYU Langone and NYU Grossman School of Medicine, New York, New York
,
Peter Izmirly
2   Division of Rheumatology, Department of Medicine, NYU Langone Health and NYU Grossman School of Medicine, New York, New York
,
Jill P. Buyon
2   Division of Rheumatology, Department of Medicine, NYU Langone Health and NYU Grossman School of Medicine, New York, New York
,
Colin K. L. Phoon
1   Division of Pediatric Cardiology, Department of Pediatrics, Hassenfeld Children's Hospital at NYU Langone and NYU Grossman School of Medicine, New York, New York
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Abstract

Objective Maternal anti-Ro (SSA) and/or anti-La (SSB) antibodies are a risk factor for congenital complete heart block (CHB). Because detailed analysis of the incidence of CHB after 24 weeks of gestational age (GA) is lacking, we aimed to ascertain the risk of “later-onset” CHB among offspring of SSA/SSB-positive mothers in the published literature.

Study Design Using search terms “neonatal lupus heart block” and “autoimmune congenital heart block” on PubMed and Ovid, we gathered prospective studies of SSA/SSB-positive mothers with fetal echo surveillance starting from before CHB diagnosis and retrospective cases of fetal CHB diagnosis after 24 weeks of GA (if there was prior normal heart rate) or after birth.

Results Ten prospective studies included 1,248 SSA/SSB-positive pregnancies with 24 cases of CHB diagnosed during pregnancy (1.9%). Among these, three (12.5%) were after 24 weeks—at weeks 25, 26, and 28. Our retrospective studies revealed 50 patients with CHB diagnosis in late fetal life and neonatal period and 34 in the nonneonatal childhood period. An additional four cases were diagnosed after age 18 years.

Conclusion Later-onset autoimmune CHB in offspring of SSA/SSB-positive mothers does occur. Our analysis suggests that prenatal surveillance should continue beyond 24 weeks of GA but is limited by inconsistent published surveillance data.

Keywords

neonatal lupus - fetal echocardiography - congenital heart block - complete heart block


Publication History

Received: 30 June 2022

Accepted: 14 March 2023

Article published online:
08 May 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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