Abstract

Fetal congenital heart block (CHB) is the most commonly observed type of fetal bradycardia, and is potentially life-threatening. More than 50% of cases of bradycardia are associated with maternal autoimmunity, and these are collectively termed immune-associated bradycardia. Several methods have been used to achieve reliable prenatal diagnoses of CHB. Emerging data and opinions on pathogenesis, prenatal diagnosis, fetal intervention, and the prognosis of fetal immune-associated CHB provide clues for generating a practical protocol for clinical management. The prognosis of fetal immune-associated bradycardia is based on the severity of heart blocks. Morbidity and mortality can occur in severe cases, thus hieratical management is essential in such cases. In this review, we mainly focus on optimal strategies pertaining to autoimmune antibodies related to CHB, although the approaches for managing autoimmune-mediated CHB are still controversial, particularly with regard to whether fetuses benefit from transplacental medication administration. To date there is still no accessible clinical strategy for autoimmune-mediated CHB. This review first discusses integrated prenatal management strategies for the condition. It then provides some advice for clinicians involved in management of fetal cardiovascular disorder.

Highlights

  • Congenital heart block (CHB) is one of the most commonly observed types of fetal bradycardia, and it entails potentially life-threatening problems for the fetus, and can result in fetal developmental delay and even intrauterine death [1]

  • We mainly focus on optimal strategies pertaining to autoimmune antibodies related to CHB, the approaches for managing autoimmune-mediated CHB are still controversial, with regard to whether fetuses benefit from transplacental medication administration

  • CHB is usually diagnosed between 18 and 24 gestational weeks (GWs) during pregnancy via fetal echocardiography techniques, but in some cases it has reportedly been identified at 16 GWs

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Summary

Introduction

Congenital heart block (CHB) is one of the most commonly observed types of fetal bradycardia, and it entails potentially life-threatening problems for the fetus, and can result in fetal developmental delay and even intrauterine death [1]. GWs, gestational weeks; AVB, atrioventricular block; PD: pulsed-wave Doppler echocardiography; MV-Ao, from the intersection of the mitral E- and A-waves to the onset of the ventricular ejection wave in the aortic outflow; SVC-Ao, from the beginning of the retrograde venous a-wave in the SVC to the beginning of the aortic ejection wave; TDI, tissue Doppler imaging, echo: echocardiography; Aa-Sa, between atrial contraction and ventricular systole; FKCG, fetal kinetocariogram, SLE, systemic lupus erythematosus; UAS, undifferentisted autoimmune syndrome; asym., asymptomatic; SS, Sjögren syndrome; CTD, connective tissue disease; MCTD, mixed CTD; Dex., dexamethasone; Bet., betamethasone; Pre., prednisone; echo, echocardiogram; HCQ, hydroxychloroquine; IVIG, intravenous immune globulin; FGR, fetal growth restriction; LBW, low birth weight.

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