Congenital Complete Atrioventricular Block With Anti–Sjögren Syndrome–Related Antigen A Antibody–Impaired Myocardium: A Case Report

CJC Pediatric and Congenital Heart Disease 2026 Open access

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Authors: Hitomi Ono, Yuichiro Takahashi, Ryuichi Shimaoka, Masako Matsui, Kazuhiko Asai, Shigenori Iwagaki, Masaki Katayama
DOI: 10.1016/j.cjcpc.2026.01.004
Cited by: 0
References: 5

Abstract

Congenital complete atrioventricular block (CAVB) occurs in 1%-2% of pregnancies positive for Sjögren syndrome–related antigen A (Ro/SSA) and B (La/SSB) antibodies.1 We report a case of CAVB with extensive impairment of the impulse-conducting myocardium in an infant born at 21 weeks 2 days to an anti-SSA–positive mother with Sjögren syndrome. Pathologic examination revealed widespread damage to the myocardium. Immunohistochemistry (IHC) showed strong anti-Ro52 staining in the affected myocardium with colocalization of maternal IgG, which also cross-reacted with fetal anti-Ro52.

Fields of Study & Subjects

Cardiac electrophysiology and arrhythmias Systemic Lupus Erythematosus Research Viral Infections and Immunology Research

Publication details

Journal / source
CJC Pediatric and Congenital Heart Disease
Volume
5
Issue
4
Pages
242–245
Publication date
18 February 2026
Language
EN