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Twenty patients with congenital complete heart block (CHB) were monitored with ECG tape recordings while awake and asleep. Episodes of marked ventricular slowing during sleep (R-R greater than 3000 msec., i.e. less than 20 beats/min) were noted in 35% (7/20). Most of these sudden R-R prolongations were 2:1 or 3:1 exit block of the junctional focus. Atrial and ventricular rate changes were mostly concordant in 2/3 but the remainder demonstrated fixed ventricular rates while atrial rates varied normally, suggesting a "sick" or "lazy" junctional focus. Other arrhythmias were found in 60% (12/20), including several types of rhythms in three patients. One child with previously unsuspected superimposed multiple arrhythmias later had a syncopal episode despite proven supra-Hisian block. The multiple patterns of atrial and ventricular rate changes found indicate complex feedback mechanisms, suggesting that congenital CHB is not a single entity. Since a significant number of patients demonstrated one or another of these unexpected and potentially hazardous findings, we recommend that congenital block patients be carefully studied, including Holter monitoring done on a regular basis.

Multiple arrhythmias detected during nocturnal monitoring in patients with congenital complete heart block.