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)]

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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

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