Do All Children with Congenital Complete Atrioventricular Block Require Permanent Pacing ?

Balmer, Christian and Bauersfeld, Urs (2003) Do All Children with Congenital Complete Atrioventricular Block Require Permanent Pacing ? [Journal (Paginated)]

Full text available as:

[img] HTML


With an incidence of 1 in 20’000 live born infants1, congenital complete atrioventricular block (CCAVB) is a rare disease. The aetiology is not completely understood. However, CCAVB may be isolated or combined with congenital heart diseases in up to 53% of affected individuals2. Isolated CCAVB is in up to 98% of the children associated with positive autoimmune antibodies in the maternal serum (anti-Ro/SS-A and anti-LA/SS-B)3,4. Interestingly, these antibodies are not specifically directed against the conduction system but also against normal myocardial cells and may cause myocarditis5,6. Affection of the conduction system can occur at different levels7. Histologically, the atrioventricular node tissue may be replaced by fibrous fatty tissue with variable involvement of the distal conduction system8. The onset of clinical symptoms in patients with CCAVB is already antenatally in up to 28%9, but can also occur only later in life. This is due to a variable degree of heart block and heart rate. Most of the symptoms are related to the slow heart rate: hydrops foetalis, heart failure of the neonate, exercise intolerance of the child. Longer pauses may cause praesyncope, syncope (classical Adams Stokes attacks) or even sudden cardiac death. Whether or not Cardiomegaly is mainly the result of a chronic compensatory increased stroke volume secondary to the slow heart rate is somewhat controversial10. Cardiomegaly may also be a distinct disease in a subgroup of patients because it does not necessarily resolve with pacemaker (PM) therapy11,12. Morbidity and mortality of CCAVB do not seem to correlate with antibody status or associated cardiac lesions13,14. There are case reports, that fetal CCAVB can be improved with steroids15. Rarely, CCAVB resolves spontaneously16,17,18,19. In most patients, the degree of conduction abnormality will either persist or worsen over time.

Item Type:Journal (Paginated)
Keywords:heart block, congenital, pacemaker, artificial, lupus erythematosus
Subjects:JOURNALS > Indian Pacing and Electrophysiology Journal
ID Code:4281
Deposited By: Indian Pacing and Electrophysiology, Journal
Deposited On:24 Apr 2005
Last Modified:11 Mar 2011 08:55

References in Article

Select the SEEK icon to attempt to find the referenced article. If it does not appear to be in cogprints you will be forwarded to the paracite service. Poorly formated references will probably not work.

1. Michaelsson M, Allen Engle M. Congenital complete heart block: an international study of the natural history. Cardiovasc Clinics 1972;4:85-101.

2. 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;91:1360-6.

3. Smeenk RJ. Immunological aspects of congenital atrioventricular block. Pacing Clin Electrophysiol 1997;20:2093-7.

4. Watson RM, Lane AT, Barnett NK, Bias WB, Arnett FC, Provost TT. Neonatal lupus erythematosus. A clinical, serological and immunogenetic study with review of the literature. Medicine 1984;63:362-8.

5. Taylor PV, Scott JS, Gerlis LM, Esscher E, Scott O. Maternal antibodies against fetal cardiac antigens in congenital complete heart block. N Engl J Med 1986;315:667-72.

6. Litsey SE, Noonan JA, O’Connor WN, Cottrill CM, Mitchell B. Maternal connective tissue disease and congenital heart block. Demonstration of immunoglobulin in cardiac tissue. N Engl J Med 1985;312:98-100.

7. Lev M. Pathogenesis of congenital atrioventricular block. Prog Cardiovasc Dis 1972;25:145-157.

8. Chow LT, Cook AC, Ho SY, Leung MP, Anderson RH. Isolated congenitally complet heart block attributable to combined nodoventricular and intraventricular discontinuity. Hum Pathol 1998;29:729-36.

9. Jaeggi ET, Hamilton RM, Silverman ED, Zamora SA, Homberger LK. Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. J Am Coll Cardiol 2002;39:130-7.

10. Kertesz NJ, Friedman RA, Colan SD, Walsh EP, Gajarski RJ, Gray PS. Left ventricular mechanics and geometry in patients with congenital complete atrioventricular block. Circulation 1997;97:3430-35.

11. Moak JP, Barron KS, Hougen TJ, Wiles HB, Balaji S, Sreeram N et al. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequel. J Am Coll Cardiol 2001;37:238-42.

12. Udink ten Cate FEA, Breur JMP, Cohen MI, Boramanand N, Kapusta L, Crosson JE et al. Dilated Cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children. J Am Coll Cardiol 2001;37:1129-34.

13. Friedman RA, Fenrich AL, Kertesz NJ. Congenital complete atrioventricular block. Pacing Clin Electrophysiol 2001;24:1681-8

14. 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 2002;4(4):345-9.

15. Copel JA, Buyon JP, Kleinman CS. Successful in utero therapy of fetal heart block. Am J Obstetr Gynecol 1995;173:1384-90.

16. Tsuji A, Yanai J, Komay T, Sato M, Asaishi T, Fukuda T. Recovery from congenital complete atrioventricular block. Pediatr Cardiol 1988;9:163-6.

17. Esscher EB. Congenital complete heart block in adolescence and adult life. A follow up study. Eur Heart J 1981;2:281-8.

18. Campbell M, Emanuel R. Six cases of congenital complete heart block followed for 34-40 years. Brit Heart J 1967;29:577-87.

19. Michaelsson M, Jonzon A, Riesenfeld T. Isolated complete atrioventricular block in adult life, a prospective study. Circulation 1995;92:442-9.

20. Gregoratos, G, Abrahams J, Epstein AE, Friedman RA, Hayes DL, Hlatky MA et al. ACC/AHA/NASPE 2002 Guideline update for implantation of cardiac pacemakers and antiarrhythmia devices – summary article. J Am Coll Cardiol 2002;40:1703-19.

21. Esscher E, Michaelsson M. QT interval in congenital complete heart block. Pediatr Cardiol 1983;4:121-4.

22. 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;316:835-9.

23. Breur JMPJ, Udink ten Kate FEA, Kapusta L, Cohen MI, Crosson JE, Boramanand N et al. Pacemaker therapy in isolated congenital complete atrioventricular block. Pacing Clin Electrophysiol 2002;25:1685-1691.

24. Villain E, Martelli H, Bonnet D, Iserin L, Butera G, Kachaner J. Characteristics and results of epicardial pacing in neonates and infants. Pacing Clin Electrophysiol 2000;23:2052-2056.

25. Bauersfeld U, Nowak B, Molinari L, Malm T, Kampmann C, Schonbeck MH, Schuller H. Low energy epicardial pacing in children: the benefit of autocapture. Ann Thorac Surg 1999;68:1380-3.


Repository Staff Only: item control page