--- abstract: | 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. altloc: - http://www.ipej.org/0303/balmer.htm chapter: ~ commentary: ~ commref: ~ confdates: ~ conference: ~ confloc: ~ contact_email: ~ creators_id: [] creators_name: - family: Balmer given: Christian honourific: '' lineage: '' - family: Bauersfeld given: Urs honourific: '' lineage: '' date: 2003-07 date_type: published datestamp: 2005-04-24 department: ~ dir: disk0/00/00/42/81 edit_lock_since: ~ edit_lock_until: ~ edit_lock_user: ~ editors_id: [] editors_name: - family: Singh given: Balbir honourific: '' lineage: '' - family: Lokhandwala given: Yash honourific: '' lineage: '' - family: Francis given: Johnson honourific: '' lineage: '' - family: Gupta given: Anup honourific: '' lineage: '' eprint_status: archive eprintid: 4281 fileinfo: /style/images/fileicons/text_html.png;/4281/1/balmer.htm full_text_status: public importid: ~ institution: ~ isbn: ~ ispublished: pub issn: ~ item_issues_comment: [] item_issues_count: 0 item_issues_description: [] item_issues_id: [] item_issues_reported_by: [] item_issues_resolved_by: [] item_issues_status: [] item_issues_timestamp: [] item_issues_type: [] keywords: 'heart block, congenital, pacemaker, artificial, lupus erythematosus' lastmod: 2011-03-11 08:55:59 latitude: ~ longitude: ~ metadata_visibility: show note: ~ number: 3 pagerange: 178-183 pubdom: TRUE publication: Indian Pacing and Electrophysiology Journal publisher: Indian Pacing and Electrophysiology Group refereed: TRUE referencetext: |- 1. 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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. relation_type: [] relation_uri: [] reportno: ~ rev_number: 8 series: ~ source: ~ status_changed: 2007-09-12 16:58:00 subjects: - ipej succeeds: ~ suggestions: ~ sword_depositor: ~ sword_slug: ~ thesistype: ~ title: "Do All Children with Congenital Complete Atrioventricular Block Require Permanent Pacing ?\n" type: journalp userid: 4424 volume: 3