Recurrent Congenital Heart Block Due to Maternal Anti-Ro Antibodies: Successful Prevention of Poor Pregnancy Outcome with Hydroxychloroquine and Added Dexamethasone

Brindusa Cimpoca-Raptis,Anca Maria Panaitescu,Nicolae Gica,Alexandra Bouariu,Anca Marina Ciobanu,Ana Maria Scutelnicu,Mihaela Popescu

doi:10.3390/reprodmed3010004

Abstract

Autoimmune Congenital Heart Block (CHB) is an immune-mediated disease caused by transplacental passage of maternal circulating anti-Ro/SSA and anti-La/SSB antibodies which can bind to fetal cardiac tissue, damaging conduction tissues by inflammation and fibrosis. Approximately 2% of pregnancies with positive anti-Ro antibodies will be complicated by fetal atrioventricular block and the risk of recurrence in subsequent pregnancies is 10 times higher. We report a case of a clinically asymptomatic patient diagnosed with anti-Ro antibodies who had two pregnancies complicated by CHB with different outcomes. Despite preventive treatment with hydroxychloroquine (HCQ) from 6 weeks of pregnancy onward, the fetus developed second to third degree CHB. Dexamethasone was added. The pregnancy evolved to near-term with persistent intermittent CHB. It is not clear how pregnancies with recurrent fetal CHB despite prophylaxis with HCQ should be managed and there is a need for controlled studies to answer the remaining questions in relation to this subject.

Highlights

Autoimmune-mediated congenital heart block (CHB), caused in the majority of cases by the transplacental passage of Anti-Ro/Anti-La maternal antibodies, is relatively rare, affecting 1 in 20,000 live births; it has a high mortality rate, up to 20% and a high morbidity rate, requiring a pacemaker implantation in most of the cases [1]
Anti-Ro/La antibodies are present in the context of some autoimmune maternal diseases, mostly related to Sjogren’s syndrome (SS) and systemic lupus erythematosus (SLE) but can be present in 2–3% of healthy, asymptomatic women and are first detected during a pregnancy complicated by CHB [2,3,4]
Transplacental immunoglobulin G (IgG) passage is an important mechanism in neonatal passive immunity and protection in the first weeks of life, there are some harmful antibodies linked to autoimmune maternal disease that are able to cross the placenta and affect the unborn baby [6]

Summary

Introduction

Autoimmune-mediated congenital heart block (CHB), caused in the majority of cases by the transplacental passage of Anti-Ro/Anti-La maternal antibodies, is relatively rare, affecting 1 in 20,000 live births; it has a high mortality rate, up to 20% and a high morbidity rate, requiring a pacemaker implantation in most of the cases [1]. Anti-Ro/La antibodies are present in the context of some autoimmune maternal diseases, mostly related to Sjogren’s syndrome (SS) and systemic lupus erythematosus (SLE) but can be present in 2–3% of healthy, asymptomatic women and are first detected during a pregnancy complicated by CHB [2,3,4]. These antibodies cause inflammation and fibrosis in the fetal cardiac muscles and atrioventricular (AV) conduction system, causing various degrees of AV block: from first-degree block, which is a benign ECG finding, being found in 6% of normal neonates, to the most severe, irreversible form, the third degree (complete) AV block. The 5 months follow-up showed no signs of ventricular dysfunction and there was no need for a pacemaker implantation

Findings

Discussion

Conclusions