Overview
Congenital heart block (CHB) is a rare congenital arrhythmia characterized by atrioventricular conduction disturbances, often associated with maternal anti-Ro/SSA and anti-La/SSB autoantibodies, leading to potential severe morbidity and mortality if untreated. 11531Diagnosis
Management
Special Populations
Key Recommendations
References
Showing 100 most recent of 196 indexed papers.
1 Pannain GD, Andrade JQ, Lopes MAB, Camargo FM, Krebs VLJ, de Carvalho WB et al.. Association between maternal anti-Ro and anti-La antibody levels and congenital heart block: a 20-year cohort study. Archives of gynecology and obstetrics 2026. link 2 Cortez D. Dual Chamber Aveir Retrievable Leadless Pacemaker Implant via the Right Internal Jugular Vein in a 13-Year-Old With Congenital Complete Heart Block. Pacing and clinical electrophysiology : PACE 2025. link 3 Kirk BP, Hale B, Karimi M. Subxiphoid Insertion of a Pediatric Implantable Pulse Generator in a Neonate With Congenital Complete Heart Block. World journal for pediatric & congenital heart surgery 2025. link 4 Weinreb SJ, Ampah SB, Okunowo O, Griffis H, Vetter VL. Longitudinal echocardiographic parameters before and after pacemaker placement in congenital complete heart block. Heart rhythm 2024. link 5 Anand AB, Kulkarni AA, Jaju GD, Sabnis GR, Mahajan AU. Temporary pacing through umbilical venous route for neonatal heart failure due to complete atrioventricular block. Pacing and clinical electrophysiology : PACE 2024. link 6 Navarre BM, Clouthier KL, Ji X, Taylor A, Weldy CS, Dubin AM et al.. miR Profile of Chronic Right Ventricular Pacing: a Pilot Study in Children with Congenital Complete Atrioventricular Block. Journal of cardiovascular translational research 2023. link 7 Gebara SA, Melov SJ, Alahakoon TI, Sholler G, Nayyar R. Fetal heart block: Vaginal delivery an option. The Australian & New Zealand journal of obstetrics & gynaecology 2023. link 8 Taşar M, Yaman ND, Arıcı B, Aksoy ÖN, Dursin H, Ayaz A et al.. Minimally invasive permanent pacemaker implantation immediately after birth: from delivery room to heart surgery. Cardiology in the young 2022. link 9 Hernstadt H, Regan W, Bhatt H, Rosenthal E, Meau-Petit V. Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period. European journal of pediatrics 2022. link 10 Wada YS, Hama I, Goto M, Ito Y, Murashima A. Long-term physical and neurodevelopmental outcomes after antenatal betamethasone administration for congenital heart block prevention. The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 2022. link 11 Weinreb SJ, Okunowo O, Griffis H, Vetter V. Incidence of morbidity and mortality in a cohort of congenital complete heart block patients followed over 40 years. Heart rhythm 2022. link 12 Gozar L, Marginean C, Fagarasan A, Muntean I, Cerghit-Paler A, Miklosi D et al.. Congenital complete atrioventricular block from literature to clinical approach - a case series and literature review. Medical ultrasonography 2021. link 13 Song MK, Kim NY, Bae EJ, Kim GB, Kwak JG, Kim WH et al.. Long-term Follow-up of Epicardial Pacing and Left Ventricular Dysfunction in Children With Congenital Heart Block. The Annals of thoracic surgery 2020. link 14 Manolis AA, Manolis TA, Melita H, Manolis AS. Congenital heart block: Pace earlier (Childhood) than later (Adulthood). Trends in cardiovascular medicine 2020. link 15 Wainwright B, Bhan R, Trad C, Cohen R, Saxena A, Buyon J et al.. Autoimmune-mediated congenital heart block. Best practice & research. Clinical obstetrics & gynaecology 2020. link 16 Kwak JG, Cho S, Kim WH. Surgical Outcomes of Permanent Epicardial Pacing in Neonates and Young Infants Less Than 1 Year of Age. Heart, lung & circulation 2019. link 17 Evers PD, Alsaied T, Anderson JB, Cnota JF, Divanovic AA. Prenatal heart block screening in mothers with SSA/SSB autoantibodies: Targeted screening protocol is a cost-effective strategy. Congenital heart disease 2019. link 18 Miyasato-Isoda M, Waguri M, Yamada Y, Miyano A, Wada Y. Anti-Ro52 antibody level is an important marker of fetal congenital heart block risk in anti-Ro/SSA antibody positive pregnancy. Modern rheumatology 2018. link 19 Tongprasert F, Sittiwangkul R, Lerthiranwong T, Tongsong T. Prenatal sonographic monitoring of progressive cardiac damages caused by anti-Ro antibodies: A case report. Journal of clinical ultrasound : JCU 2018. link 20 Bason C, Pagnini I, Brucato A, Maestroni S, Puccetti A, Lunardi C et al.. Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response. Lupus 2017. link 21 Cabrera Ortega M, Duamy HT, Benítez Ramos DB. Key Role of Pacing Site as Determinant Factor of Exercise Testing Performance in Pediatric Patients with Chronic Ventricular Pacing. Pediatric cardiology 2017. link 22 Cuneo BF, Moon-Grady AJ, Sonesson SE, Levasseur S, Hornberger L, Donofrio MT et al.. Heart sounds at home: feasibility of an ambulatory fetal heart rhythm surveillance program for anti-SSA-positive pregnancies. Journal of perinatology : official journal of the California Perinatal Association 2017. link 23 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. Modern rheumatology 2016. link 24 Tingström J, Hjelmstedt A, Welin Henriksson E, Ambrosi A, Sonesson SE, Wahren-Herlenius M. Anti-Ro/SSA autoantibody-positive women's experience of information given on the risk of congenital heart block. Lupus 2016. link 25 Clancy RM, Markham AJ, Reed JH, Blumenberg M, Halushka MK, Buyon JP. Targeting downstream transcription factors and epigenetic modifications following Toll-like receptor 7/8 ligation to forestall tissue injury in anti-Ro60 associated heart block. Journal of autoimmunity 2016. link 26 Silvetti MS, Di Carlo D, Ammirati A, Placidi S, Di Mambro C, Ravà L et al.. Left ventricular pacing in neonates and infants with isolated congenital complete or advanced atrioventricular block: short- and medium-term outcome. Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology 2015. link 27 Motonaga KS, Punn R, Axelrod DM, Ceresnak SR, Hanisch D, Kazmucha JA et al.. Diminished exercise capacity and chronotropic incompetence in pediatric patients with congenital complete heart block and chronic right ventricular pacing. Heart rhythm 2015. link 28 Beake MJ, Bhole V, Johnston T, Rasiah SV. Successful emergency cardiac pacing and permanent pacemaker insertion in a preterm 29-week gestation hydropic baby with congenital complete heart block. Cardiology in the young 2015. link 29 Sacks JH, Samai C, Gomez K, Kanaan U. Maternal antibody-associated fetal second-degree heart block and atrial flutter: case report and review. Pediatric cardiology 2013. link 30 Tingström J, Henriksson EW, Sonesson SE, Wahren-Herlenius M. Ro52 autoantibody-positive women's experience of being pregnant and giving birth to a child with congenital heart block. Midwifery 2013. link 31 Bordachar P, Zachary W, Ploux S, Labrousse L, Haissaguerre M, Thambo JB. Pathophysiology, clinical course, and management of congenital complete atrioventricular block. Heart rhythm 2013. link 32 Anami A, Fukushima K, Takasaki Y, Sumida T, Waguri M, Wake N et al.. The predictive value of anti-SS-A antibodies titration in pregnant women with fetal congenital heart block. Modern rheumatology 2013. link 33 Ambrosi A, Salomonsson S, Eliasson H, Zeffer E, Skog A, Dzikaite V et al.. Development of heart block in children of SSA/SSB-autoantibody-positive women is associated with maternal age and displays a season-of-birth pattern. Annals of the rheumatic diseases 2012. link 34 Ambrosi A, Dzikaite V, Park J, Strandberg L, Kuchroo VK, Herlenius E et al.. Anti-Ro52 monoclonal antibodies specific for amino acid 200-239, but not other Ro52 epitopes, induce congenital heart block in a rat model. Annals of the rheumatic diseases 2012. link 35 Ruffatti A, Milanesi O, Chiandetti L, Cerutti A, Gervasi MT, De Silvestro G et al.. A combination therapy to treat second-degree anti-Ro/La-related congenital heart block: a strategy to avoid stable third-degree heart block?. Lupus 2012. link 36 Kwak JG, Kim SJ, Song JY, Choi EY, Lee SY, Shim WS et al.. Permanent epicardial pacing in pediatric patients: 12-year experience at a single center. The Annals of thoracic surgery 2012. link 37 Koh C, Hong WJ, Yung TC, Lun KS, Wong SJ, Cheung YF. Left ventricular torsional mechanics and diastolic function in congenital heart block with right ventricular pacing. International journal of cardiology 2012. link 38 Miyoshi T, Maeno Y, Sago H, Inamura N, Yasukohchi S, Kawataki M et al.. Evaluation of transplacental treatment for fetal congenital bradyarrhythmia: – nationwide survey in Japan –. Circulation journal : official journal of the Japanese Circulation Society 2012. link 39 Serikawa T, Ichikawa K, Kikuchi A, Takakuwa K, Tanaka K. A case study of a pregnant patient with a congenital heart block accompanied by left isomerism and uncontrolled type 2 diabetes who was treated successfully with ritodrine. Gynecologic and obstetric investigation 2010. link 40 Sharma G, Linden MD, Schultz DS, Inamdar KV. Cystic tumor of the atrioventricular node: an unexpected finding in an explanted heart. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2010. link 41 Karnabi E, Qu Y, Wadgaonkar R, Mancarella S, Yue Y, Chahine M et al.. Congenital heart block: identification of autoantibody binding site on the extracellular loop (domain I, S5-S6) of alpha(1D) L-type Ca channel. Journal of autoimmunity 2010. link 42 Hayashi T, Kaneko M, Kim KS, Eryu Y, Shindo T, Isoda T et al.. Outcome of prenatally diagnosed isolated congenital complete atrioventricular block treated with transplacental betamethasone or ritodrine therapy. Pediatric cardiology 2009. link 43 Matsubara S, Morimatsu Y, Shiraishi H, Kuwata T, Ohkuchi A, Izumi A et al.. Fetus with heart failure due to congenital atrioventricular block treated by maternally administered ritodrine. Archives of gynecology and obstetrics 2008. link 44 Chen CA, Chang CI, Wang JK, Lin MT, Chiu SN, Chiu HH et al.. Restoration of cardiac function by setting the ventricular pacing at a lower range in an infant with congenital complete atrioventricular block and dilated cardiomyopathy. International journal of cardiology 2008. link 45 Takasugi H, Watanabe K, Ono Y, Sakaguchi H, Motoki N, Yoshida Y et al.. Myocardial scintigraphy after pacemaker implantation for congenital complete atrioventricular block. European journal of pediatrics 2008. link 46 Radbill AE, Brown DW, Lacro RV, Cecchin F, Berul CI, Triedman JK et al.. Ascending aortic dilation in patients with congenital complete heart block. Heart rhythm 2008. link 47 Clancy RM, Zheng P, O'Mahony M, Izmirly P, Zavadil J, Gardner L et al.. Role of hypoxia and cAMP in the transdifferentiation of human fetal cardiac fibroblasts: implications for progression to scarring in autoimmune-associated congenital heart block. Arthritis and rheumatism 2007. link 48 Monem A, Chohan U, Ali M. Epidural anaesthesia during labour for a patient with congenital complete heart block: a case report. JPMA. The Journal of the Pakistan Medical Association 2007. link 49 Tissot C, Aggoun Y, Rimensberger PC, Sierra J, Kalangos A, Beghetti M et al.. Left ventricular epicardial VVI pacing for a congenital complete heart block with severe myocardial dysfunction: shall epicardial pacing wires be positioned left?. International journal of cardiology 2007. link 50 Filippi L, Vangi V, Murzi B, Moschetti R, Colella A. Temporary epicardial pacing in an extremely low-birth-weight infant with congenital atrioventricular block. Congenital heart disease 2007. link 51 Spence D, Hornberger L, Hamilton R, Silverman ED. Increased risk of complete congenital heart block in infants born to women with hypothyroidism and anti-Ro and/or anti-La antibodies. The Journal of rheumatology 2006. link 52 Chan JB, Levi DS, Lai CK, Alejos JC, Fishbein MC. Cellular rejection of the conduction system after orthotopic heart transplantation for congenital atrioventricular block. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2006. link 53 Wahren-Herlenius M, Sonesson SE. Specificity and effector mechanisms of autoantibodies in congenital heart block. Current opinion in immunology 2006. link 54 Stevens AM, Hermes HM, Lambert NC, Nelson JL, Meroni PL, Cimaz R. Maternal and sibling microchimerism in twins and triplets discordant for neonatal lupus syndrome-congenital heart block. Rheumatology (Oxford, England) 2005. link 55 Kussman BD, Madril DR, Thiagarajan RR, Walsh EP, Laussen PC. Anesthetic management of the neonate with congenital complete heart block: a 16-year review. Paediatric anaesthesia 2005. link 56 Neufing PJ, Clancy RM, Jackson MW, Tran HB, Buyon JP, Gordon TP. Exposure and binding of selected immunodominant La/SSB epitopes on human apoptotic cells. Arthritis and rheumatism 2005. link 57 Clancy RM, Buyon JP. Autoimmune-associated congenital heart block: dissecting the cascade from immunologic insult to relentless fibrosis. The anatomical record. Part A, Discoveries in molecular, cellular, and evolutionary biology 2004. link 58 Li Z, Strasburger JF, Cuneo BF, Gotteiner NL, Wakai RT. Giant fetal magnetocardiogram P waves in congenital atrioventricular block: a marker of cardiovascular compensation?. Circulation 2004. link 59 Rakza T, Rifai N, Delapintiere A, Magnenant E, Vaksmann G, Bonnevalle M et al.. Electro-stimulation in preterm neonates with congenital auriculo-ventricular block. Report of three cases. European journal of pediatrics 2004. link 60 Clancy RM, Buyon JP. Clearance of apoptotic cells: TGF-beta in the balance between inflammation and fibrosis. Journal of leukocyte biology 2003. link 61 Buyon JP, Friedman DM. Autoantibody-associated congenital heart block: the clinical perspective. Current rheumatology reports 2003. link 62 Novi JM, Mulvihill BH. Use of a subcutaneous beta-sympathomimetic pump for the treatment of fetal congenital complete heart block. A case report. The Journal of reproductive medicine 2003. link 63 Costedoat-Chalumeau N, Amoura Z, Le Thi Hong D, Wechsler B, Vauthier D, Ghillani P et al.. Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block. Annals of the rheumatic diseases 2003. link 64 Mohan VK, Naik AK, Bharti N, Shende D. A patient with congenital complete heart block undergoing multiple exposures to general anaesthesia. Anaesthesia and intensive care 2003. link 65 Stevens AM, Hermes HM, Rutledge JC, Buyon JP, Nelson JL. Myocardial-tissue-specific phenotype of maternal microchimerism in neonatal lupus congenital heart block. Lancet (London, England) 2003. link14795-2) 66 Clancy RM, Backer CB, Yin X, Kapur RP, Molad Y, Buyon JP. Cytokine polymorphisms and histologic expression in autopsy studies: contribution of TNF-alpha and TGF-beta 1 to the pathogenesis of autoimmune-associated congenital heart block. Journal of immunology (Baltimore, Md. : 1950) 2003. link 67 von Schnakenburg C, Fink C, Peuster M, Wessel A, Vazquez-Jimenez JF. Permanent pacemaker implantation in a 1,445 g preterm neonate on the first day of life. The Thoracic and cardiovascular surgeon 2002. link 68 Breur JM, Udink Ten Cate FE, Kapusta L, Cohen MI, Crosson JE, Boramanand N et al.. Pacemaker therapy in isolated congenital complete atrioventricular block. Pacing and clinical electrophysiology : PACE 2002. link 69 Minassian VA, Jazayeri A. Favorable outcome in a pregnancy with complete fetal heart block and severe bradycardia. Obstetrics and gynecology 2002. link02071-9) 70 Matsushita H, Higashino M, Sekizuka N, Kurabayashi T, Takakuwa K, Tanaka K. Successful prenatal treatment of congenital heart block with ritodrine administered transplacentally. Archives of gynecology and obstetrics 2002. link 71 Balmer C, Fasnacht M, Rahn M, Molinari L, Bauersfeld U. Long-term follow up of children with congenital complete atrioventricular block and the impact of pacemaker therapy. Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology 2002. link 72 Di Donato F, Chan EK, Askanase AD, Miranda-Carus M, Buyon JP. Interaction between 52 kDa SSA/Ro and deubiquitinating enzyme UnpEL: a clue to function. The international journal of biochemistry & cell biology 2001. link00055-3) 73 Eronen M, Heikkilä P, Teramo K. Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment, and outcome in six cases. Pediatric cardiology 2001. link 74 Domanović D, Zavrsnik T, Vesel S. Autologous placental blood transfusion after a planned neonatal pacemaker implantation. Transfusion medicine (Oxford, England) 2001. link 75 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. Gynecologic and obstetric investigation 2001. link 76 Tseng JJ, Chou MM, Ho ES. Perinatal management of congenital complete heart block. Zhonghua yi xue za zhi = Chinese medical journal; Free China ed 2000. link 77 Villain E, Martelli H, Bonnet D, Iserin L, Butera G, Kachaner J. Characteristics and results of epicardial pacing in neonates and infants. Pacing and clinical electrophysiology : PACE 2000. link 78 Brackley KJ, Ismail KM, Wright JG, Kilby MD. The resolution of fetal hydrops using combined maternal digoxin and dexamethasone therapy in a case of isolated complete heart block at 30 weeks gestation. Fetal diagnosis and therapy 2000. link 79 Watanabe H, Hayashi J, Sugawara M, Hashimoto T, Sato S, Takeuchi K. Cardiac strangulation in a neonatal case: a rare complication of permanent epicardial pacemaker leads. The Thoracic and cardiovascular surgeon 2000. link 80 Yamada H, Kato EH, Ebina Y, Moriwaki M, Yamamoto R, Furuta I et al.. Fetal treatment of congenital heart block ascribed to anti-SSA antibody: case reports with observation of cardiohemodynamics and review of the literature. American journal of reproductive immunology (New York, N.Y. : 1989) 1999. link 81 Saleeb S, Copel J, Friedman D, Buyon JP. Comparison of treatment with fluorinated glucocorticoids to the natural history of autoantibody-associated congenital heart block: retrospective review of the research registry for neonatal lupus. Arthritis and rheumatism 1999. link42:11<2335::AID-ANR12>3.0.CO;2-3) 82 Menon A, Silverman ED, Gow RM, Hamilton RM. Chronotropic competence of the sinus node in congenital complete heart block. The American journal of cardiology 1998. link00569-4) 83 Fukushige J, Takahashi N, Igarashi H, Nakayama H, Inoue K, Hijii T. Perinatal management of congenital complete atrioventricular block: report of nine cases. Acta paediatrica Japonica : Overseas edition 1998. link 84 Miranda ME, Tseng CE, Rashbaum W, Ochs RL, Casiano CA, Di Donato F et al.. Accessibility of SSA/Ro and SSB/La antigens to maternal autoantibodies in apoptotic human fetal cardiac myocytes. Journal of immunology (Baltimore, Md. : 1950) 1998. link 85 Uerpairojkit B, Charoenvidhya D, Chottivittayatanakorn P, Tannirandorn Y, Benjachollamas W, Phaosavasdi S. Fetal complete heart block: an expectant management. Journal of the Medical Association of Thailand = Chotmaihet thangphaet 1998. link 86 McLeod KA, Rankin AC, Houston AB. 1:1 atrioventricular conduction in congenital complete heart block. Heart (British Cardiac Society) 1998. link 87 Julkunen H, Kaaja R, Siren MK, Mack C, McCready S, Holthöfer H et al.. Immune-mediated congenital heart block (CHB): identifying and counseling patients at risk for having children with CHB. Seminars in arthritis and rheumatism 1998. link80042-5) 88 Henneveld H, Hutter P, Bink-Boelkens M, Sreeram N. Junctional ectopic tachycardia evolving into complete heart block. Heart (British Cardiac Society) 1998. link 89 Begg L, East C, Chan FY, Brennecke S. Intrapartum fetal oxygen saturation monitoring in congenital fetal heart block. The Australian & New Zealand journal of obstetrics & gynaecology 1998. link 90 Rosenthal D, Druzin M, Chin C, Dubin A. A new therapeutic approach to the fetus with congenital complete heart block: preemptive, targeted therapy with dexamethasone. Obstetrics and gynecology 1998. link00149-5) 91 Miranda-Carús ME, Boutjdir M, Tseng CE, DiDonato F, Chan EK, Buyon JP. Induction of antibodies reactive with SSA/Ro-SSB/La and development of congenital heart block in a murine model. Journal of immunology (Baltimore, Md. : 1950) 1998. link 92 Smeenk RJ. Immunological aspects of congenital atrioventricular block. Pacing and clinical electrophysiology : PACE 1997. link 93 Sherman SJ, Featherstone LS. Congenital complete heart block and successful vaginal delivery. Journal of perinatology : official journal of the California Perinatal Association 1997. link 94 Michaelsson M, Riesenfeld T, Jonzon A. Natural history of congenital complete atrioventricular block. Pacing and clinical electrophysiology : PACE 1997. link 95 Rosenthal E, Bostock J. VDD pacing in children with congenital complete heart block: advantages of a single pass lead. Pacing and clinical electrophysiology : PACE 1997. link 96 Kertesz NJ, Friedman RA, Colan SD, Walsh EP, Gajarski RJ, Gray PS et al.. Left ventricular mechanics and geometry in patients with congenital complete atrioventricular block. Circulation 1997. link 97 Horsfall AC, Li JM, Maini RN. Placental and fetal cardiac laminin are targets for cross-reacting autoantibodies from mothers of children with congenital heart block. Journal of autoimmunity 1996. link 98 Agarwala B, Sheikh Z, Cibils LA. Congenital complete heart block. Journal of the National Medical Association 1996. link 99 Michaëlsson M, Jonzon A, Riesenfeld T. Isolated congenital complete atrioventricular block in adult life. A prospective study. Circulation 1995. link 100 Copel JA, Buyon JP, Kleinman CS. Successful in utero therapy of fetal heart block. American journal of obstetrics and gynecology 1995. link90621-5)