关键词: AV node Calcification Heart block Lupus

Mesh : Humans Female Atrioventricular Block / immunology congenital diagnosis etiology physiopathology Pregnancy Adult Infant, Newborn Atrioventricular Node / physiopathology pathology Calcinosis / immunology pathology Fatal Outcome Autoimmune Diseases / immunology diagnosis complications Autopsy Antibodies, Antinuclear / immunology blood Autoantibodies / immunology blood Autoimmunity Heart Block / congenital

来  源:   DOI:10.1016/j.carpath.2024.107634

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.
摘要:
母亲自身免疫性疾病是先天性心脏传导阻滞(CHB)的最常见原因,一种罕见的疾病,其特征是由于母体自身抗体抗SSA/Ro和抗SSB/La引起的胎儿房室(AV)结纤维化和钙化。我们报告了一名房室结高度房室传导阻滞和钙化的女性新生儿的完整尸检和临床信息,房室结的心房入路,以及左右束分支,生于一名27岁女性,患有亚临床自身免疫性疾病。
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