Use of Intravenous Gamma Globulin and Corticosteroids in the Treatment of Maternal Autoantibody-Mediated Cardiomyopathy

Abstract

Treatment of maternal autoantibody-mediated fetal cardiomyopathy/endocardial fibroelastosis with IVIG and corticosteroids potentially improves the outcome of affected fetuses. Further studies are needed to determine the optimal dose and timing of IVIG administration.

Neonatal Lupus Erythematosus

“…However, the effectiveness of these agents in the treatment of endocardial fibroelastosis is unknown [41]. In a retrospective study for the use of glucocorticoids and IVIG (both in utero and after birth), a potential survival benefit was observed in cases with cardiomyopathy/endocardial fibroelastosis based upon comparison to historical control data [86].…”

Almanac 2012: Congenital heart disease. The national society journals present selected research that has driven recent advances in clinical cardiology

“…14 In a non-randomised multi-centre study of 20 foetuses exposed to maternal lupus antibodies 15 it was found that treatment with intravenous gamma globulin and steroids potentially improved the outcome for these children, with better than expected survival. However, a prospective study of 165 foetuses with exposure to anti-Ro/La antibody found that foetal atrioventricular prolongation did not predict progression to heart block, so management based on the strategy of identifying and treating foetal atrioventricular prolongation was questioned.…”

Fetal Echocardiography and Prenatal Cardiovascular Interventions – An Update (2012)

“…With earlier detection of more subtle atrioventricular block in the context of maternal autoimmune mediated fetal heart disease, we have begun to better understand the clinical evolution of this disease, its association with other arrhythmias such as junctional ectopic and ventricular tachycardia, and sinus node dysfunction and more diffuse myocardial disease, and to explore strategies to improve the survival of affected fetuses [51][52][53][54][55][56].…”

“…Initial interest in earlier assessment stemmed from the advent of endovaginal transducers in the late 1980s which permit imaging in closer proximity to the fetus at less than 14 weeks of gestation through the maternal cervix [56][57][58]. Recognition that transabdominal imaging with high frequency transducers permits assessment of many pregnancies at earlier ages has also resulted in greater enthusiasm for early screening among pediatric cardiologists who have not been trained and/or are uncomfortable with transvaginal imaging [60][61][62].…”

“…To date, although there is no treatment that consistently reverses or prevents atrioventricular block in the context of maternal auto-antibodies SSA/Ro and SSB/La, our eventual goal in this disease, corticosteroids have been used to reduce myocardial inflammation and sympathomimetic therapies have been used to increase the fetal heart rate and improve myocardial performance with variable success [42,51,54]. More recently, recognition of the presence of associated myocardial disease has led to successful treatment with intravenous gamma globulin and corticosteroids in utero and early infancy [56]. Larger prospective randomized studies are necessary at this time to provide further evidence of the benefit of such a regiment that exposes the "healthy" mother (70-80% of whom have no clinical autoimmune disease) to blood products (intravenous gammaglobulin).…”