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Autoimmune congenital heart block: complex and unusual situations
P Brito-Zerón1, P M Izmirly2, M Ramos-Casals1
1Josep Font Laboratory of Autoimmune Diseases, Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Lupus
|January 15, 2016
Summary
Autoimmune congenital heart block (ACHB), caused by maternal antibodies, leads to severe heart conduction issues in newborns. This review explores complex cases and risks associated with ACHB, impacting fetal development and infant survival.
Area of Science:
- Cardiology
- Immunology
- Neonatology
Background:
- Autoimmune congenital heart block (ACHB) is a serious condition in neonatal lupus.
- Maternal Ro/La autoantibodies crossing the placenta damage fetal heart conduction.
- It can cause irreversible complete atrioventricular (AV) block, cardiomyopathy, and other cardiac issues.
Purpose of the Study:
- To review complex and unusual clinical scenarios of ACHB.
- To analyze various risk factors and complications associated with ACHB.
- To discuss management strategies and outcomes in affected infants.
Main Methods:
- Literature review of clinical cases and studies on ACHB.
- Analysis of maternal immunological profiles and autoimmune diseases.
- Examination of outcomes including mortality, pacemaker implantation, and transplant needs.
Main Results:
- ACHB presents with high mortality (20%) and pacemaker rates (64%).
- Associated conditions include endocardial fibroelastosis and cardiomyopathies.
- Unusual presentations involve diverse maternal factors, assisted reproductive techniques, and multiple births.
Conclusions:
- ACHB poses significant risks to newborns, necessitating specialized care.
- Understanding complex ACHB cases is crucial for improved diagnosis and management.
- Further research into maternal factors and novel risks like IVF is warranted.
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