Background Congenital heart block (CHB) risk is 1–2% in case of maternal anti SSA/Ro and/or anti SSB/La antibody positivity. CHB have significant mortality (20–30%) and available therapeutic options' efficacy is contradictory.
Objectives To review the literature regarding different treatment modalities for CHB.
Methods We performed a systematic review (August 2015) on Pubmed, using the following MeSH terms: “neonatal lupus”, “congenital heart block”; results were restricted to human studies and English language. 1125 articles were assessed in abstract form and, after employing exclusion criteria, 267 original articles/case reports were evaluated in full text. Finally, 199 studies were included, reporting on a total of 492 CHB patients. All administered treatments were assessed on a patient-by-patient basis.
Results A total of 243 cases reported data for CHB treatment: glucocorticoids (GCs) in 106 (43.6%) cases, intravenous immunoglobulin (IVIG) in 14 (5.7%) cases, and hydroxychloroquine in 5 (2.0%) cases. 21 patients received plasmapheresis treatment. 134 (55.1%) cases received no treatment. Both GCs and IVIG were mostly used in cases with complete atrioventricular (AV) block (74.1% and 61.5% of cases, respectively).
Different types of GCs were used: Dexamethasone in 54 (58.6%) patients, prednisolone in 27 (29.3%) and betamethasone in 11 (11.9%) patients (total 92 patients with available data). Dosing schemes and regimens were also widely heterogeneous, with fifteen different regimens used by different centres (Table). Regarding IVIG treatment, six different algorithms were used. Similarly, five different plasmapheresis protocols were used.
Conclusions There is no consensus in the treatment of CHB. Drug selection and dosing regimens have wide heterogeneity. More than half cases received no treatment. Of note, prednisolone has often been used, despite its inability to cross the placenta.
Disclosure of Interest None declared