Treatment and follow-up of fetuses that developed congenital heart block due to autoantibody in two cases

Abstract

Objective Autoantibody-related congenital heart block (ACHB) is a passively acquired autoimmune disease. This study aimed to examine the pathogenesis, clinical manifestations, and treatment of ACHB. Method The clinical data of two fetuses with first-degree ACHB were retrospectively analyzed. Results Two pregnant women were strongly positive for anti-Sjögren’s syndrome-related antigen A (SSA) antibody. Among these two cases, one had a prolonged atrioventricular (AV) interval at 28+3 weeks in utero, while the other had a prolonged AV interval at 24+6 weeks in utero. After prenatal intervention, one patient recovered to normal, while one fetus continued to have ACHB after treatment with dexamethasone and intravenous immunoglobulin. Furthermore, the two neonates were positive for anti-SSA antibody and were diagnosed with ACHB. Conclusion The pathogenesis of ACHB is closely correlated with anti-SSA/Ro antibody and anti-SSB/La antibody from the mother, and is affected by fetal susceptibility. Early screening and early intervention for ACHB are important.

Echogenic Fetal Heart Without Conduction Defect in Maternal Autoimmune Disease: A Lesser Known Association

“…Due to the small number of cases with different maternal therapy methods, it is not possible to comment on the association between maternal variables and postnatal outcomes. However, as steroids have been shown to improve in-utero survival in fetal heart blocks [13], we wonder if it had a role in our cases too. In the case of postnatal mortality, we could only presume the cause of death to be due to the detrimental effect of tachycardia, myocarditis or both as the demise happened immediately after birth and detailed evaluation was not performed.…”

Plasmapheresis in Neonatal Lupus

“…Additional investigation into single and double agents show varied data; small to medium sized studies describing the use of plasmapheresis and IVIG in at-risk pregnant mothers have been performed. Combination treatments with IVIG and steroids alone have at least been shown to revert the clinically diagnosed in utero CHB in a case report [76]. As single agents, plasmapheresis, IVIG, and immunoadsorption are presumed to decrease serum SSA and SSB antibodies that can cross the placenta to the fetus.…”