22. Rosen KM, Mehta A, Rahimtoola SH, Miller RA. Sites of congenital and surgical heart block as defined by His bundle electrocardiography. Circulation 1971; 44:833. https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 12/20 7/5/23, 10:35 AM Congenital third-degree (complete) atrioventricular block - UpToDate 23. Feldt RH, DuShane JW, Titus JL. The atrioventricular conduction system in persistent common atrioventricular canal defect: correlations with electrocardiogram. Circulation 1970; 42:437. 24. Lev M, Silverman J, Fitzmaurice FM, et al. Lack of connection between the atria and the more peripheral conduction system in congenital atrioventricular block. Am J Cardiol 1971; 27:481. 25. Lev M, Cuadros H, Paul MH. Interruption of the atrioventricular bundle with congenital atrioventricular block. Circulation 1971; 43:703. 26. James TN, Spencer MS, Kloepfer JC. De Subitaneis Mortibus. XXI. Adult onset syncope. with comments on the nature of congenital heart block and the morphogenesis of the human atrioventricular septal junction. Circulation 1976; 54:1001. 27. Reid JM, Coleman EN, Doig W. Complete congenital heart block. Report of 35 cases. Br Heart J 1982; 48:236. 28. Nasrallah AT, Gillette PC, Mullins CE. Congenital and surgical atrioventricular block within the His bundle. Am J Cardiol 1975; 36:914. 29. Sholler GF, Walsh EP. Congenital complete heart block in patients without anatomic cardiac defects. Am Heart J 1989; 118:1193. 30. Alexander E, Buyon JP, Provost TT, Guarnieri T. Anti-Ro/SS-A antibodies in the pathophysiology of congenital heart block in neonatal lupus syndrome, an experimental model. In vitro electrophysiologic and immunocytochemical studies. Arthritis Rheum 1992; 35:176. 31. Miranda-Car s ME, Askanase AD, Clancy RM, et al. Anti-SSA/Ro and anti-SSB/La autoantibodies bind the surface of apoptotic fetal cardiocytes and promote secretion of TNF-alpha by macrophages. J Immunol 2000; 165:5345. 32. Garcia S, Nascimento JH, Bonfa E, et al. Cellular mechanism of the conduction abnormalities induced by serum from anti-Ro/SSA-positive patients in rabbit hearts. J Clin Invest 1994; 93:718. 33. Xiao GQ, Hu K, Boutjdir M. Direct inhibition of expressed cardiac l- and t-type calcium channels by igg from mothers whose children have congenital heart block. Circulation 2001; 103:1599. 34. Cruz RB, Viana VS, Nishioka SA, et al. Is isolated congenital heart block associated to neonatal lupus requiring pacemaker a distinct cardiac syndrome? Pacing Clin Electrophysiol 2004; 27:615. https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 13/20 7/5/23, 10:35 AM Congenital third-degree (complete) atrioventricular block - UpToDate 35. Askanase AD, Friedman DM, Copel J, et al. Spectrum and progression of conduction abnormalities in infants born to mothers with anti-SSA/Ro-SSB/La antibodies. Lupus 2002; 11:145. 36. Nield LE, Silverman ED, Taylor GP, et al. Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis. Circulation 2002; 105:843. 37. Eliasson H, Sonesson SE, Salomonsson S, et al. Outcome in young patients with isolated complete atrioventricular block and permanent pacemaker treatment: A nationwide study of 127 patients. Heart Rhythm 2015; 12:2278. 38. Waltuck J, Buyon JP. Autoantibody-associated congenital heart block: outcome in mothers and children. Ann Intern Med 1994; 120:544. 39. Pinsky WW, Gillette PC, Garson A Jr, McNamara DG. Diagnosis, management, and long-term results of patients with congenital complete atrioventricular block. Pediatrics 1982; 69:728. 40. Dewey RC, Capeless MA, Levy AM. Use of ambulatory electrocardiographic monitoring to identify high-risk patients with congenital complete heart block. N Engl J Med 1987; 316:835. 41. Micha lsson M, Jonzon A, Riesenfeld T. Isolated congenital complete atrioventricular block in adult life. A prospective study. Circulation 1995; 92:442. 42. Karpawich PP, Gillette PC, Garson A Jr, et al. Congenital complete atrioventricular block: clinical and electrophysiologic predictors of need for pacemaker insertion. Am J Cardiol 1981; 48:1098. 43. Kusumoto FM, Schoenfeld MH, Barrett C, et al. 2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol 2019; 74:e51. 44. Epstein AE, DiMarco JP, Ellenbogen KA, et al. 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol 2013; 61:e6. 45. Writing Committee Members, Shah MJ, Silka MJ, et al. 2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients. Heart Rhythm 2021; 18:1888. 46. Baruteau AE, Pass RH, Thambo JB, et al. Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management. Eur J Pediatr 2016; 175:1235. https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 14/20 7/5/23, 10:35 AM Congenital third-degree (complete) atrioventricular block - UpToDate 47. Khairy P, Landzberg MJ, Gatzoulis MA, et al. Transvenous pacing leads and systemic thromboemboli in patients with intracardiac shunts: a multicenter study. Circulation 2006; 113:2391. 48. Chubb H, Mah D, Dubin AM, Moore J. Conduction system pacing in pediatric and congenital heart disease. Front Physiol 2023; 14:1154629. 49. Izmirly PM, Saxena A, Kim MY, et al. Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus. Circulation 2011; 124:1927. 50. Levesque K, Morel N, Maltret A, et al. Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome. Autoimmun Rev 2015; 14:1154. 51. Hernstadt H, Regan W, Bhatt H, et al. Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period. Eur J Pediatr 2022; 181:1047. 52. McHenry MM. Factors influencing longevity in adults with congenital complete heart block. Am J Cardiol 1972; 29:416. 53. Reybrouck T, Vanden Eynde B, Dumoulin M, Van der Hauwaert LG. Cardiorespiratory response to exercise in congenital complete atrioventricular block. Am J Cardiol 1989; 64:896. 54. Motonaga KS, Punn R, Axelrod DM, et al. Diminished exercise capacity and chronotropic incompetence in pediatric patients with congenital complete heart block and chronic right ventricular pacing. Heart Rhythm 2015; 12:560. 55. Kertesz NJ, Friedman RA, Colan SD, et al. Left ventricular mechanics and geometry in patients with congenital complete atrioventricular block. Circulation 1997; 96:3430. 56. Moak JP, Barron KS, Hougen TJ, et al. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela. J Am Coll Cardiol 2001; 37:238. 57. Udink ten Cate FE, Breur JM, Cohen MI, et al. Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children. J Am Coll Cardiol 2001; 37:1129. 58. Pordon CM, Moodie DS. Adults with congenital complete heart block: 25-year follow-up. Cleve Clin J Med 1992; 59:587. 59. Groves AM, Allan LD, Rosenthal E. Outcome of isolated congenital complete heart block diagnosed in utero. Heart 1996; 75:190. 60. 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; https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 15/20 7/5/23, 10:35 AM Congenital third-degree (complete) atrioventricular block - UpToDate 19:1149. 61. Thambo JB, Bordachar P, Garrigue S, et al. Detrimental ventricular remodeling in patients with congenital complete heart block and chronic right ventricular apical pacing. Circulation 2004; 110:3766. 62. Chandler SF, Fynn-Thompson F, Mah DY. Role of cardiac pacing in congenital complete heart block. Expert Rev Cardiovasc Ther 2017; 15:853. 63. Dandamudi G, Simon J, Cano O, et al. Permanent His Bundle Pacing in Patients With Congenital Complete Heart Block: A Multicenter Experience. JACC Clin Electrophysiol 2021; 7:522. Topic 912 Version 35.0 https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 16/20 7/5/23, 10:35 AM Congenital third-degree (complete) atrioventricular block - UpToDate GRAPHICS Anatomy of a normal heart and L-transposition of the great arteries RA: right atrium; RV: right ventricle; Ao: aorta; LV: left ventricle; LA: left atrium; PT: pulmonary trunk. Graphic 83845 Version 1.0 https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 17/20 7/5/23, 10:35 AM Congenital third-degree (complete) atrioventricular block - UpToDate Different forms of atrioventricular canal defects Anatomic and physiologic similarities between the different forms of AVSDs are illustrated. Complete AVSDs have 1 annulus with large interatrial and interventricular communications. Intermediate defects (1 annulus, 2 orifices) are a subtype of complete AVSD. Complete AVSDs have physiology of VSDs and ASDs. In contrast, partial AVSDs have physiology of ASDs. Transitional defects are a form of complete AVSD in which a small, insignificant inlet VSD is present and, as a result, the physiology is more similar to that of a partial defect. Partial defects and the intermediate form of complete AVSD share a similar anatomic feature: A tongue of tissue divides the common AV valve into distinct right and left orifices. LA: left atrium; LPV: left pulmonary vein; LV: left ventricle; RA: right atrium; RPV: right pulmonary vein; RV: right ventricle; VSD: ventricular septal defect; ASD: atrial septal defect; AV: atrioventricular; AVSD: atrioventricular septal defect. Reproduced with permission from: Cetta F, Minich LL, Edwards WD, et al. Atrioventricular septal defects. In: Moss and Adams' Heart Disease in Infants, Children, and Adolescents Including the Fetus and Young Adult, 7th ed, Allen HD, Shaddy RE, Driscoll DJ, Feltes TF (Eds), Lippincott Williams & Wilkins, Philadelphia 2007. Copyright 2007 Lippincott Williams & Wilkins. www.lww.com. Graphic 86346 Version 9.0 https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 18/20 7/5/23, 10:35 AM Congenital third-degree (complete) atrioventricular block - UpToDate Timing of pacemaker implantation according to age at diagnosis of congenital CHB In a study of 102 cases of congenital complete heart block (CHB), most patients ultimately had a pacemaker inserted, regardless of the time of onset of the syndrome. By 20 years of age, only 11 percent of neonatal and 12 percent of childhood cases had not required pacemaker implantation. Data from Jaeggi ET, Hamilton RM, Silverman ED, et al, J Am Coll Cardiol 2002; 39:130. Graphic 55309 Version 2.0 https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 19/20 7/5/23, 10:35 AM Congenital third-degree (complete) atrioventricular block - UpToDate Contributor Disclosures William H Sauer, MD Grant/Research/Clinical Trial Support: Biosense-Webster [Catheter ablation]; Boston Scientific [Catheter ablation]. All of the relevant financial relationships listed have been mitigated. Edward P Walsh, MD No relevant financial relationship(s) with ineligible companies to disclose. Mark S Link, MD No relevant financial relationship(s) with ineligible companies to disclose. Susan B Yeon, MD, JD, FACC No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/congenital-third-degree-complete-atrioventricular-block/print 20/20