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Autoimmune-mediated congenital heart block
Benjamin Wainwright1, Rohit Bhan1, Catherine Trad1
1NYU School of Medicine, New York, NY, USA.
Best Practice & Research. Clinical Obstetrics & Gynaecology
|November 6, 2019
Summary
Autoimmune congenital heart block (CHB) in neonates results from maternal antibodies damaging the fetal heart's AV node. This irreversible condition requires understanding its classification, pathogenesis, and management.
Area of Science:
- Cardiology
- Immunology
- Neonatology
Background:
- Autoimmune-mediated congenital heart block (CHB) is a severe manifestation of neonatal lupus.
- It involves passive transfer of maternal anti-SSA/Ro and anti-SSB/La autoantibodies, causing fetal heart conduction tissue damage.
- The damage leads to inflammation and fibrosis of the atrioventricular (AV) node, occurring after fetal heart structural development.
Purpose of the Study:
- To review the classification, prevalence, pathogenesis, and clinical management of autoimmune CHB.
- To provide a comprehensive overview of this serious neonatal condition.
Main Methods:
- This is a review article, synthesizing existing literature on autoimmune CHB.
- Information was gathered on classification, prevalence, pathogenesis, and clinical management strategies.
Main Results:
- Complete AV block is the most common and irreversible manifestation of CHB.
- Other cardiac complications include endocardial fibroelastosis (EFE), dilated cardiomyopathy, and valvular insufficiency.
- The disease mechanism involves maternal autoantibodies and subsequent fetal cardiac inflammation and fibrosis.
Conclusions:
- Autoimmune CHB is a significant concern in neonatal lupus, characterized by irreversible AV block.
- Understanding the pathogenesis is crucial for developing effective clinical management strategies.
- Early recognition and management are essential for affected neonates.
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