Congenital atrioventricular block


  • José Mário Baggio Junior
  • Joubert Ariel Pereira Mosquera
  • Ewandro Luiz Rey Moura


Atrioventricular Block/Congenital, Pacemaker Artifical, Placental Circulation


Congenital complete atrioventricular block is observed in approximately 1 in 20,000 live births and is associated to increased fetal mortality and morbidity. Complete atrioventricular block may be secondary to immune or non-immune processes. Immune-mediated congenital
complete atrioventricular blocks are associated with the transplacental passage of anti-Ro/SSA and anti-La/SSB maternal antibodies. These antibodies damage the conduction tissue of susceptible fetuses. This report examines the pathophysiology of congenital complete
atrioventricular block, the role of transplacental therapy and pacemaker indication.


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1. 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(7):1658-66. DOI: org/10.1016/S0735-1097(98)00161-2.

2. Michaëlsson M, Engle MA. Congenital complete heart block: an international study of the natural history. Cardiovasc Clin. 1972;4(3):85-101.

3. Jaeggi E, Laskin C, Hamilton R, Kingdom J, Silverman E. The importance of the level of maternal anti-Ro/SSA antibodies as a prognostic marker of the development of cardiac neonatal lupus erythematosus a prospective study of 186 antibody-exposed fetuses and infants. J Am Coll Cardiol. 2010;55(24):2778-84. DOI: 10.1016/j.jacc.2010.02.042.

4. Jaeggi ET, Hamilton RM, Silverman ED, Zamora SA, Hornberger LK. 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(1):130-7. DOI: 10.1016/S0735-1097(01)01697-7.

5. Eronen M, Sirèn MK, Ekblad H, Tikanoja T, Julkunen H, Paavilainen T. Short- and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn. Pediatrics. 2000;106(1 Pt 1):86-91.

6. Schmidt KG, Ulmer HE, Silverman NH, Kleinman CS, Copel JA. Perinatal outcome of fetal complete atrioventricular block: a multicenter experience. J Am Coll Cardiol. 1991;17(6):1360-6.

7. 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(18):1927-35.
DOI: 10.1161/CIRCULATIONAHA.111.033894.

8. Berg C, Geipel A, Kohl T, et al. Atrioventricular block detected in fetal life: associated anomalies and potential prognostic markers. Ultrasound Obstet Gynecol. 2005;26(1):4-15. DOI: 10.1002/uog.1918.

9. Simpson JM. Fetal arrhythmias. Ultrasound Obstet Gynecol.
2006;27(6):599-606. DOI: 10.1002/uog.2819.

10. Allan LD, Anderson RH, Sullivan ID, Campbell S, Holt DW, Tynan M. Evaluation of fetal arrhythmias by echocardiography. Br Heart J. 1983;50(3):240-5.

11. Anderson RH, Becker AE, Arnold R, Wilkinson JL. The conducting tissues in congenitally corrected transposition. Circulation. 1974;50(5):911-23.

12. Lopes LM, Tavares GM, Damiano AP, et al. Perinatal outcome of fetal atrioventricular block: one-hundred-sixteen cases from a single institution. Circulation. 2008;118(12):1268-75. DOI: 10.1161/CIRCULATIONAHA.107.735118.

13. Friedman DM, Llanos C, Izmirly PM, et al. Evaluation of fetuses in a study of intravenous immunoglobulin as preventive therapy for congenital heart block: Results of a multicenter, prospective, open-label clinical trial. Arthritis Rheum. 2010;62(4):1138-46. DOI: 10.1002/art.27308.

14. Julkunen H, Eronen M. The rate of recurrence of isolated congenital heart block: a population-based study. Arthritis Rheum. 2001;44(2):487-8.<487::AIDANR70>3.0.CO;2-D.

15. Llanos C, Izmirly PM, Katholi M, et al. Recurrence rates of cardiac manifestations associated with neonatal lupus and maternal/fetal risk factors. Arthritis Rheum. 2009;60(10):3091-7. DOI: 10.1002/art.24768.

16. Jaeggi ET, Fouron JC, Silverman ED, Ryan G, Smallhorn J, Hornberger LK. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease. Circulation. 2004;110(12):1542-8. CIR.0000142046.58632.3A.

17. Sonesson SE, Salomonsson S, Jacobsson LA, Bremme K, Wahren-Herlenius M. Signs of first-degree heart block occur in one-third of fetuses of pregnant women with anti-SSA/Ro 52-kd antibodies. Arthritis Rheum. 2004;50(4):1253-61.

18. Copel JA, Buyon JP, Kleinman CS. Successful in utero therapy of fetal heart block. Am J Obstet Gynecol. 1995;173(5):1384-90.

19. Yamada H, Kato EH, Ebina Y, et al. Fetal treatment of congenital heart block ascribed to anti-SSA antibody: case reports with observation of cardiohemodynamics and review of the literature. Am J Reprod Immunol. 1999;42(4):226-32. DOI: 10.1111/j.1600-0897.1999.tb00095.x.

20. Eliasson H, Sonesson SE, Sharland G, 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):1919-26. DOI: 10.1161/CIRCULATIONAHA.111.041970.

21. Bloom SL, Sheffield JS, McIntire DD, Leveno KJ. Antenatal dexamethasone and decreased birth weight. Obstet Gynecol. 2001;97(4):485-90. DOI: 10.1097/00006250-200104000-00001.

22. National Institutes of Health Consensus Development Panel. Antenatal corticosteroids revisited: repeat courses - National Institutes of Health Consensus Development Conference Statement, August 17-18, 2000. Obstet Gynecol. 2001;98(1):144-50.

23. Martin TA. Congenital heart block: current thoughts on management, morphologic spectrum, and role of intervention. Cardiol Young. 2014;Suppl 2:41-6. DOI: 10.1017/S1047951114001358.

24. Motonaga KS, Dubin AM. Cardiac resynchronization therapy for pediatric patients with heart failure and congenital heart disease: a reappraisal of results. Circulation. 2014;129(18):1879-91.

25. Michaëlsson M, Jonzon A, Riesenfeld T. Isolated congenital complete atrioventricular block in adult life. A prospective study. Circulation. 1995;92(3):442-9.



How to Cite

Baggio Junior, J. M., Ariel Pereira Mosquera, J., & Rey Moura, E. L. (2017). Congenital atrioventricular block. JOURNAL OF CARDIAC ARRHYTHMIAS, 31(3), 98–101. Retrieved from



Cardiac Pacing