Autoimmune-associated Congenital Heart Block

Abstract

Objective:Congenital heart block (CHB) is a rare but life-threatening disorder. More than half of CHB cases are associated with maternal autoimmune, which are termed as autoimmune-associated CHB. This review summarized the recent research findings in understanding autoimmune-associated CHB, discussed the current diagnostic approaches and management strategies, and summarized the problems and future directions for this disorder.Data Sources:We retrieved the articles published in English from the PubMed database up to January 2017, using the keywords including “Autoimmune-associated”, “Autoimmune-mediated”, and “Congenital heart block”.Study Selection:Articles about autoimmune-associated CHB were obtained and reviewed.Results:Observational studies consistently reported that transplacental maternal antibodies might recognize fetal or neonatal antigens in various tissues and result in immunological damages, but the molecular mechanisms underlying CHB pathogenesis still need illuminated. Multiple factors were involved in the process of atrioventricular block development and progression. While several susceptibility genes had been successfully defined, how these genes and their protein interact and impact each other remains to be explored. With currently available diagnostic tools, fetal ultrasound cardiography, and fetal magnetocardiography, most of CHB could be successfully diagnosed and comprehensively evaluated prenatally. The efficacy of current approaches for preventing the progression and recurrence of CHB and other autoimmune-mediated damages was still controversial.Conclusions:This review highlighted the relationships between autoimmune injuries and CHB and strengthened the importance of perinatal management and therapy for autoimmune-associated CHB.

Foetal bradycardia due to congenital heart blocks: a study of three cases with review literature

“…Intravenous immunoglobulin and plasmapheresis have been used to decrease autoantibodies in maternal circulation to treat CHB in mothers with autoimmune diseases. Although there were possible benefits, intravenous immunoglobulin and/or plasmapheresis either did not reverse the progression to complete heart block or did not produce long term satisfactory results [21]. Hence, administration of maternal terbutaline, Dexamethasone and plasmapheresis in combination has been used in situations when the fetal demise is imminent.…”

A 9 months old child with Asymptomatic Bradycardia

“…Thus, close follow up of cardiac function, especially ventricular function, is necessary. 6 The mean 5-year survival rate of patients with CAVB, post implantation of pacemaker is about 76-89%. Long-term complications include high-grade stenosis or occlusion of the great cardiac veins (25%) as well as infectious complications.…”

BIiochemical Markers in Pregnancy Associated with Sj�gren�s Syndrome and Thrombophilia

“…Several hypotheses of the molecular mechanism leading to CHB have been proposed, one of the theories accepted being that anti-Ro52 antibody produces disturbances in signal conduction due to the direct impact on the calcium homeostasis in the fetal heart [13].…”