Congenital Complete Atrioventricular Heart Block in a Pregnant Woman with Sjögren Syndrome: Prenatal Care Follow-Up and the Challenge of Intrauterine Treatment

Abstract The present report describes a case of complete atrioventricular block (CAVB) diagnosed at 25 weeks of gestation in a pregnant woman with Sjögren's syndrome and positive anti-Ro/SSA antibodies. Fluorinated steroids (dexamethasone and betamethasone) and terbuline were used to increase the fetal heart rate, but the fetal heart block was not reversible, and the administration of drugs was discontinued due to maternal collateral effects. Follow-up fetal echocardiograms were performed, and the fetus evolved with pericardial effusion, presence of fibroelastosis in the right ventricle, and ventricular dysfunction. Interruption of pregnancy by cesarean section was indicated at 34 weeks of gestation, and a cardiac pacemaker was implanted in the male newborn immediately after birth. Therapy for fetuses with CAVB is controversial mainly regarding the use or not of corticosteroids; however, monitoring of the atrioventricular interval by fetal echocardiography should be performed in fetuses from pregnant women with positive autoantibodies anti-Ro/SSA and/or anti-La/SSB to prevent the progression to CAVB.摘要 本病例报告描述1例合并干燥综合征且抗Ro/SSA抗体（anti-Ro/SSA antibodies）阳性的孕妇，于妊娠25周时确诊完全性房室传导阻滞（complete atrioventricular block，CAVB）。研究者予氟化糖皮质激素（地塞米松与倍他米松）及特布他林以提升胎心率，但胎儿心脏传导阻滞并未逆转，且因出现母体不良反应而停药。后续对胎儿行超声心动图随访，结果提示胎儿出现心包积液、右心室纤维弹性组织增生及心室功能不全。于妊娠34周时行剖宫产终止妊娠，该男性新生儿出生后即刻植入心脏起搏器。目前针对合并CAVB的胎儿，其治疗方案尚存争议，尤以糖皮质激素的应用与否为甚；但对于抗Ro/SSA及/或抗La/SSB抗体（anti-La/SSB antibodies）阳性孕妇所孕育的胎儿，应通过胎儿超声心动图监测房室间期，以预防其进展为CAVB。