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Congenital third degree (complete) atrioventricular block

INTRODUCTION

Atrioventricular (AV) block is defined as a delay or interruption in the transmission of an impulse from the atria to the ventricles due to an anatomical or functional impairment in the conduction system. The conduction disturbance can be transient or permanent. The conduction can be delayed, intermittent, or absent. The commonly used terminology includes first degree (slowed conduction without missed beats), second degree (missed beats, often in a regular pattern, eg, 2:1, 3:2, or higher degrees of block), and third degree or complete AV block. (See "ECG tutorial: Atrioventricular block".)

Congenital third degree, or complete, heart block (CHB) was first described in 1901 by Morquio, who also noted the familial occurrence and the association with Stokes-Adams attacks and death [1]. The presence of fetal bradycardia (40 to 80 beats per minute) as a manifestation of CHB was first noted in 1921 and is the initial sign of this disorder in many cases [2]. The incidence of congenital CHB in the general population varies between 1 in 15,000 to 1 in 22,000 live-born infants [3].

The etiology, presentation, treatment, and prognosis associated with congenital CHB will be presented here. Discussions of the etiologies of acquired AV block as well as non-congenital complete AV block are presented separately. (See "Etiology of atrioventricular block" and "Third degree (complete) atrioventricular block".)

ETIOLOGY

Neonatal lupus, previously known as maternal lupus and caused by maternal antibodies that cross the placenta, is responsible for 60 to 90 percent of cases of congenital CHB overall [4-6]. It accounts for almost all cases presenting in utero or the neonatal period, but for only a few cases occurring later (5 percent in one report) [6]. (See "Neonatal lupus".)

Other causes include myocarditis and various structural cardiac defects, particularly congenitally corrected transposition of the great arteries, atrioventricular discordance, or polysplenia with atrioventricular canal defect. Several hereditary disorders have also been identified. (See 'Non-immune congenital AV block' below and "Etiology of atrioventricular block", section on 'Familial disease'.)

             

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Literature review current through: May 2013. | This topic last updated: Mar 14, 2013.
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References
Top
  1. Morquio L. Sur une maladie infantile et familiale characterisée par des modifications permanentes du pouls, des attaques syncopales et epileptiforme et la mort subite. Arch Méd d'Enfants 1901; 4:467.
  2. White P, Eustis R. Congenital heart block. Am J Dis Child 1921; 22:299.
  3. Michaëlsson M, Engle MA. Congenital complete heart block: an international study of the natural history. Cardiovasc Clin 1972; 4:85.
  4. Johansen AS, Herlin T. [Neonatal lupus syndrome. Association with complete congenital atrioventricular block]. Ugeskr Laeger 1998; 160:2521.
  5. Ross BA. Congenital complete atrioventricular block. Pediatr Clin North Am 1990; 37:69.
  6. Jaeggi ET, Hamilton RM, Silverman ED, et al. Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. A single institution's experience of 30 years. J Am Coll Cardiol 2002; 39:130.
  7. Brucato A, Frassi M, Franceschini F, et al. Risk of congenital complete heart block in newborns of mothers with anti-Ro/SSA antibodies detected by counterimmunoelectrophoresis: a prospective study of 100 women. Arthritis Rheum 2001; 44:1832.
  8. Buyon JP, Kim MY, Copel JA, Friedman DM. Anti-Ro/SSA antibodies and congenital heart block: necessary but not sufficient. Arthritis Rheum 2001; 44:1723.
  9. Buyon JP, Hiebert R, Copel J, et al. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 1998; 31:1658.
  10. Ho SY, Esscher E, Anderson RH, Michaëlsson M. Anatomy of congenital complete heart block and relation to maternal anti-Ro antibodies. Am J Cardiol 1986; 58:291.
  11. 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.
  12. Baruteau AE, Fouchard S, Behaghel A, et al. Characteristics and long-term outcome of non-immune isolated atrioventricular block diagnosed in utero or early childhood: a multicentre study. Eur Heart J 2012; 33:622.
  13. Baruteau AE, Behaghel A, Fouchard S, et al. Parental electrocardiographic screening identifies a high degree of inheritance for congenital and childhood nonimmune isolated atrioventricular block. Circulation 2012; 126:1469.
  14. Anderson RH, Wenick AC, Losekoot TG, Becker AE. Congenitally complete heart block. Developmental aspects. Circulation 1977; 56:90.
  15. NAKAMURA FF, NADAS AS. COMPLETE HEART BLOCK IN INFANTS AND CHILDREN. N Engl J Med 1964; 270:1261.
  16. ROWE JC, WHITE PD. Complete heart block: a follow-up study. Ann Intern Med 1958; 49:260.
  17. 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.
  18. Feldt RH, DuShane JW, Titus JL. The atrioventricular conduction system in persistent common atrioventricular canal defect: correlations with electrocardiogram. Circulation 1970; 42:437.
  19. 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.
  20. Lev M, Cuadros H, Paul MH. Interruption of the atrioventricular bundle with congenital atrioventricular block. Circulation 1971; 43:703.
  21. 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.
  22. Reid JM, Coleman EN, Doig W. Complete congenital heart block. Report of 35 cases. Br Heart J 1982; 48:236.
  23. Nasrallah AT, Gillette PC, Mullins CE. Congenital and surgical atrioventricular block within the His bundle. Am J Cardiol 1975; 36:914.
  24. Sholler GF, Walsh EP. Congenital complete heart block in patients without anatomic cardiac defects. Am Heart J 1989; 118:1193.
  25. 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.
  26. Miranda-Carús 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.
  27. 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.
  28. 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.
  29. 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.
  30. Nield LE, Silverman ED, Taylor GP, et al. Maternal anti-Ro and anti-La antibody-associated endocardial fibroelastosis. Circulation 2002; 105:843.
  31. Glickstein JS, Buyon J, Friedman D. Pulsed Doppler echocardiographic assessment of the fetal PR interval. Am J Cardiol 2000; 86:236.
  32. Waltuck J, Buyon JP. Autoantibody-associated congenital heart block: outcome in mothers and children. Ann Intern Med 1994; 120:544.
  33. Friedman DM, Kim MY, Copel JA, et al. Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study. Circulation 2008; 117:485.
  34. 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.
  35. Michaëlsson M, Jonzon A, Riesenfeld T. Isolated congenital complete atrioventricular block in adult life. A prospective study. Circulation 1995; 92:442.
  36. 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.
  37. 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.
  38. Esscher EB. Congenital complete heart block in adolescence and adult life. A follow-up study. Eur Heart J 1981; 2:281.
  39. McHenry MM. Factors influencing longevity in adults with congenital complete heart block. Am J Cardiol 1972; 29:416.
  40. 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.
  41. Kertesz NJ, Friedman RA, Colan SD, et al. Left ventricular mechanics and geometry in patients with congenital complete atrioventricular block. Circulation 1997; 96:3430.
  42. 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.
  43. 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.
  44. Pordon CM, Moodie DS. Adults with congenital complete heart block: 25-year follow-up. Cleve Clin J Med 1992; 59:587.
  45. Groves AM, Allan LD, Rosenthal E. Outcome of isolated congenital complete heart block diagnosed in utero. Heart 1996; 75:190.
  46. 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.
  47. Sweeney MO, Hellkamp AS, Ellenbogen KA, et al. Adverse effect of ventricular pacing on heart failure and atrial fibrillation among patients with normal baseline QRS duration in a clinical trial of pacemaker therapy for sinus node dysfunction. Circulation 2003; 107:2932.