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Complete congenital heart block: risk of occurrence and therapeutic approach to prevention
1Department of Medicine, Hospital for Joint Diseases, NYU School of Medicine, NY 10003.
Insights
High-risk pregnancies with a history of complete congenital heart block (CCHB) can be managed with maternal plasmapheresis and prednisone. This aggressive treatment reduces maternal antibodies, preventing CCHB in the fetus.
Area of Science:
- Immunology
- Maternal-Fetal Medicine
- Cardiology
Background:
- Complete congenital heart block (CCHB) poses a significant risk for subsequent pregnancies, with 41% of cases having affected siblings.
- Maternal autoantibodies, specifically anti-SSA(Ro) and anti-SSB(La), are implicated in CCHB pathogenesis.
- Identifying high-risk pregnancies is crucial for intervention.
Abstract:
A retrospective literature review revealed that in 41% of cases of complete congenital heart block (CCHB) there was at least one other affected sibling, emphasizing the considerable risk of carrying a second affected fetus with CCHB. Therefore an aggressive approach was taken to prevent CCHB in a fetus with a high risk for the condition as defined by (1) presence of DR3 and high titers of antibodies to SSA(Ro) and SSB(La) in the mother (2) previous history of CCHB in a sibling. In a feasibility study, thrice weekly plasmapheresis was initiated in the 19th week of gestation to remove antibodies from the maternal circulation in advance of major placental transport to the developing fetus. Prednisone was also administered to decrease antibody synthesis. The concentration of total maternal IgG and antibodies to SSA(Ro) and SSB(La) were decreased by greater than 60% during the course of therapy. Planned delivery of a healthy baby was done at 36 weeks.