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Histological finding of maternal antibody-associated congenital heart block accompanied by large atrial septal defect and severe cardiac dysfunction

Published online by Cambridge University Press:  30 March 2022

Yasuhiro Endo
Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan
Yoko Kawai*
Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan
Masaaki Yamagishi
Department of Pediatric Cardiovascular Surgery, Children’s Medical Center, Kyoto Prefectural University of Medicine, Kyoto, Japan
Author for correspondence: Yoko Kawai, MD, Department of Pediatrics, Kyoto Prefectural University of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, Japan. Tel: +81-75-251-5571; Fax: +81-75-252-1399. E-mail:


Cardiac dysfunction commonly occurs in congenital heart block associated with maternal anti-SSA antibodies, especially after pacemaker implantation. We report the case of a 4-year-old girl with antibody-associated congenital heart block and a large secundum atrial septal defect who presented with significant cardiac dysfunction 4 years after pacemaker implantation. Histological findings were useful for determining the course of treatment and perioperative risk of intracardiac repair.

Brief Report
© The Author(s), 2022. Published by Cambridge University Press

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