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

Cardiovasc Pathol. 2024 Sep-Oct:72:107634. doi: 10.1016/j.carpath.2024.107634. Epub 2024 Mar 18.

Abstract

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.

Keywords: AV node; Calcification; Heart block; Lupus.

Publication types

  • Case Reports
  • Review

MeSH terms

  • 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
  • Calcinosis / immunology
  • Calcinosis / pathology
  • Fatal Outcome
  • Female
  • Heart Block / congenital
  • Humans
  • Infant, Newborn
  • Pregnancy

Substances

  • Antibodies, Antinuclear
  • Autoantibodies

Supplementary concepts

  • Congenital heart block