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.

Resumo

Este relato descreve um caso de bloqueio atrioventricular completo (BAVC) diagnosticado com 25 semanas de gestação em uma mulher com síndrome de Sjögren e anticorpos anti-Ro/SSA positivos. Esteroides fluoretados (dexametasona e betametasona) e terbulina foram utilizados para aumentar a frequência cardíaca fetal, mas o bloqueio cardíaco fetal não foi reversível, e a administração dos medicamentos foi interrompida devido a efeitos colaterais maternos. Ecocardiogramas fetais de acompanhamento foram realizados, e o feto evoluiu com derrame pericárdico, presença de fibroelastose no ventrículo direito, e disfunção ventricular. A interrupção da gravidez por cesariana foi indicada com 34 semanas, e um marca-passo cardíaco foi implantado no recém-nascido do sexo masculino imediatamente após o nascimento. A terapia para fetos com BAVC é controversa, principalmente no que diz respeito ao uso ou não de corticosteroides; no entanto, o monitoramento do intervalo atrioventricular pela ecocardiografia fetal deve ser feito em fetos de mulheres grávidas com autoanticorpos positivos anti-Ro/SSA e/ou anti-La/SSB para impedir a progressão para o BAVC.

Keywords

complete congenital heart block - maternal autoantibodies - prenatal diagnosis - prevention - intrauterine treatment

Palavras-chave

bloqueio cardíaco congênito completo - autoanticorpos maternos - diagnóstico pré-natal - prevenção - tratamento intrauterino

• References

• 1 Bordachar P, Zachary W, Ploux S, Labrousse L, Haissaguerre M, Thambo JB. , et al. Pathophysiology, clinical course, and management of congenital complete atrioventricular block. Heart Rhythm 2013; 10 (05) 760-766 . Doi: 10.1016/j.hrthm.2012.12.030
• 2 Pedra SRFF, Zielinsky P, Binotto CN, Martins CN, Fonseca ESVB, Guimarães ICB. , et al. Brazilian Fetal Cardiology Guidelines - 2019. Arq Bras Cardiol 2019; 112 (05) 600-648 . Doi: 10.5935/abc.20190075
• 3 Donofrio MT, Moon-Grady AJ, Hornberger LK, Copel JA, Sklansky MS, Abuhamad A. , et al; American Heart Association Adults With Congenital Heart Disease Joint Committee of the Council on Cardiovascular Disease in the Young and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and Council on Cardiovascular and Stroke Nursing. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation 2014; 129 (21) 2183-2242 . Doi: 10.1161/01.cir.0000437597.44550.5d
• 4 Gupta S, Gupta N. Sjögren syndrome and pregnancy: a literature review. Perm J 2017; 21: 16-047 . Doi: 10.7812/TPP/16-047
• 5 Tsuboi H, Sumida T, Noma H, Yamagishi K, Anami A, Fukushima K. , et al. Maternal predictive factors for fetal congenital heart block in pregnant mothers positive for anti-SS-A antibodies. Mod Rheumatol 2016; 26 (04) 569-575 . Doi: 10.3109/14397595.2015.1106661
• 6 Zemlin M, Bauer K, Dörner T, Altinöz H, Versmold H. [Intrauterine therapy and outcome in four pregnancies of one mother with anti ro-autoantibody positive Sjoegren's syndrome]. Z Geburtshilfe Neonatol 2002; 206 (01) 22-25 . Doi: 10.1055/s-2002-20947. German.
• 7 Aoki H, Inamura N, Kawazu Y, Nakayama M, Kayatani F. Fetal echocardiographic assessment of endocardial fibroelastosis in maternal anti-SSA antibody-associated complete heart block. Circ J 2011; 75 (05) 1215-1221 . Doi: 10.1253/circj.cj-10-1032
• 8 Nield LE, Silverman ED, Taylor GP, Smallhorn JF, Mullen JBM, Silverman NH. , et al. Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis. Circulation 2002; 105 (07) 843-848 . Doi: 10.1161/hc0702.104182
• 9 Eliasson H, Sonesson SE, Sharland G, Granath F, Simpson J, Carvalho JS. , et al; Fetal Working Group of the European Association of Pediatric Cardiology. Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients. Circulation 2011; 124 (18) 1919-1926 . Doi: 10.1161/CIRCULATIONAHA.111.041970
• 10 Lopes LM, Tavares GMP, Damiano AP, Lopes MAB, Aiello VD, Schultz R. , et al. Perinatal outcome of fetal atrioventricular block: one-hundred-sixteen cases from a single institution. Circulation 2008; 118 (12) 1268-1275 . Doi: 10.1161/CIRCULATIONAHA.107.735118
• 11 Pike JI, Donofrio MT, Berul CI. Ineffective therapy, underpowered studies, or merely too little, too late? Risk factors and impact of maternal corticosteroid treatment on outcome in antibody-associated fetal heart block. Circulation 2011; 124 (18) 1905-1907 . Doi: 10.1161/CIRCULATIONAHA.111.061267
• 12 Yoshida H, Iwamoto M, Sakakibara H, Shigeta H, Hirahara F, Sato K. Treatment of fetal congenital complete heart block with maternal administration of beta-sympathomimetics (terbutaline): a case report. Gynecol Obstet Invest 2001; 52 (02) 142-144 . Doi: 10.1159/000052960
• 13 Hofstaetter C, Hansmann M, Eik-Nes SH, Huhta JC, Luther SL. A cardiovascular profile score in the surveillance of fetal hydrops. J Matern Fetal Neonatal Med 2006; 19 (07) 407-413 . Doi: 10.1080/14767050600682446
• 14 Gardiner HM. In utero intervention for severe congenital heart disease. Best Pract Res Clin Obstet Gynaecol 2019; 58: 42-54 . Doi: 10.1016/j.bpobgyn.2019.01.007
• 15 Rein AJJT, Mevorach D, Perles Z, Gavri S, Nadjari M, Nir A. , et al. Early diagnosis and treatment of atrioventricular block in the fetus exposed to maternal anti-SSA/Ro-SSB/La antibodies: a prospective, observational, fetal kinetocardiogram-based study. Circulation 2009; 119 (14) 1867-1872 . Doi: 10.1161/CIRCULATIONAHA.108.773143
• 16 Andreoli L, Bertsias GK, Agmon-Levin N, Brown S, Cervera R, Costedoat-Chalumeau N. , et al. EULAR recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Ann Rheum Dis 2017; 76 (03) 476-485 . Doi: 10.1136/annrheumdis-2016-209770