Subclinical maternal autoimmune disease leading to congenital high degree atrioventricular block: Case report and review of the literature

• Published in: Cardiovascular pathology 2024-09, Vol.72, p.107634, Article 107634
• Main Authors: Walcott, Shelby E., Tan, Christian H., Wicker, Jason, Litovsky, Silvio
• Format: Article
• Language: English
• Subjects: Adult; Antibodies, Antinuclear - blood; Antibodies, Antinuclear - immunology; Atrioventricular Block - congenital; Atrioventricular Block - diagnosis; Atrioventricular Block - etiology; Atrioventricular Block - immunology; Atrioventricular Block - physiopathology; Atrioventricular Node - pathology; Atrioventricular Node - physiopathology; Autoantibodies - blood; Autoantibodies - immunology; Autoimmune Diseases - complications; Autoimmune Diseases - diagnosis; Autoimmune Diseases - immunology; Autoimmunity; Autopsy; AV node; Calcification; Calcinosis - immunology; Calcinosis - pathology; Fatal Outcome; Female; Heart block; Heart Block - congenital; Humans; Infant, Newborn; Lupus; Pregnancy
• ISSN: 1054-8807; 1879-1336; 1879-1336
• DOI: 10.1016/j.carpath.2024.107634

•Subclinical autoimmune disease leading to high degree atrioventricular block.•Extensive fibrosis, calcification, and fat infiltration of the AV conduction system.•Extensive calcification without inflammation in the AV node and bundle branches. Maternal autoimmune disease is the most common cause of congenital heart block (CHB), a rare illness characterized by fibrosis and calcification of the fetal atrioventricular (AV) node due to maternal autoantibodies anti-SSA/Ro and anti-SSB/La. We report the full autopsy and clinical information on a female neonate with high degree AV block and calcification in the AV node, atrial approaches to the AV node, and both right and left bundle branches, born to a 27-year-old female with subclinical autoimmune disease.