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Reversible complete atrioventricular block in a neonate with maternal anti-Sjögren’s syndrome-associated antibody: atypical phenotype of autoimmune congenital heart block

Published online by Cambridge University Press:  19 July 2023

Hirofumi Saiki Open the ORCID record for Hirofumi Saiki [Opens in a new window] ,
Kanchi Saito  and
Akira Sato
Hirofumi Saiki*
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Iwate Medical University, Yahaba, Japan
Kanchi Saito
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Iwate Medical University, Yahaba, Japan
Akira Sato
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Iwate Medical University, Yahaba, Japan
*
Corresponding author: H. Saiki; Email: hirosaiki-circ@umin.ac.jp
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Abstract

The fetus of anti-Sjögren’s syndrome-associated antibody-positive mother developed complete atrioventricular block at 39 weeks of gestation and required urgent ventricular pacing after birth. Unexpectedly, the patient recovered from the atrioventricular block within a few days. Fraction analysis of maternal anti-Sjögren’s syndrome-associated antibody revealed positivity for isolated anti-Ro/SSA 60 kDa antibody, which is abnormal as most patients with complete atrioventricular block present with anti-Ro/SSA 52 kDa positivity, which may indicate a potentially atypical late and reversible manifestation of an autoimmune congenital atrioventricular block in this patient.

Keywords

Fetal atrioventricular blockmaternal anti-Sjögren’s syndrome-associated antibodyneonatal loops syndromeimmune congenital atrioventricular block
Type
Brief Report
Information
Cardiology in the Young , Volume 33 , Issue 11 , November 2023 , pp. 2430 - 2433
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Copyright
© The Author(s), 2023. Published by Cambridge University Press

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