Complete atrioventricular block in patients with atrioventricular discordance

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.