Circulation, Vol 67, 1374-1377, Copyright © 1983 by American Heart Association
JC Huhta, JD Maloney, DG Ritter, DM Ilstrup and RH Feldt
Although patients with atrioventricular (AV) discordance (corrected
transposition) have abnormal conduction pathways and may spontaneously
develop high-grade AV block, no quantitative assessment of the risk of this
happening is available. We reviewed the data on 107 patients with AV
discordance ages 2-76 years (mean 22 years) at follow-up. Eighty-two
patients (77%) had a ventricular septal defect, 57 (53%) had pulmonary
stenosis, 35 (34%) had tricuspid insufficiency and 24 (23%) had
dextrocardia. Twenty-three patients (22%) had complete AV block. This
condition was present in four patients at birth and developed in 19
patients at ages 4 months to 53 years (mean 18.1 years). Nine of these
patients had permanent pacemaker implantation, four at the onset of
complete block and five an average of 11 years later. Nine patients have AV
block but no pacemaker. One patient died suddenly. Detailed data analysis
showed that with increasing follow-up the risk of natural onset AV block
continued at a rate of approximately 2% per year after diagnosis. The
presence of an intact ventricular septum made AV block more likely. We
conclude that patients with AV discordance are at risk of developing
complete AV block throughout their lives. With increasing age, this risk is
approximately constant and is probably not significantly increased by
corrective or palliative surgery if acute surgical AV block does not occur.
Pacemaker implantation is not necessary in some children with spontaneous
AV block.
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Complete atrioventricular block in patients with atrioventricular discordance
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