Complete heart block with rhythmically recurring decay and failure of the idioventricular pacemaker.
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The effect of heart rate (HR) on maximum working capacity was studied in 18 patients (mean age 55 years) with a physiological pacemaker implanted for chronic complete heart block of different aetiology. Exercise testing was performed by bicycle ergometry in 3 pacing protocols in randomized sequence, and without the patients knowing the preprogrammed pacing mode: 1) VVI pacing at a rate of 30 beats/min--exercise on heart block; 2) VVI pacing at a rate of 70 beats/min; 3) physiological DDD or QT pacing. While patients with heart block had a mean HR of 54 +/- 16 beats/min and working capacity of 81 +/- 31 W, on VVI 70 pacing the values were 73 +/- 9 beats/min (+35%, p < 0.002) and 100 +/- 45 W (+24%, p < 0.008), respectively. Exercise during physiological pacing led to an increase in HR to 140 +/- 15 beats/min (+169%, p < 0.002) and in working capacity to 130 +/- 52 W (+61%, p < 0.002). Thus, the increment in working capacity was 2.5 times higher on physiological than standard ventricular pacing (p < 0.005) if compared with the working capacity of patients with heart block.
Three cases of congenitally complete heart block are described of hearts in which other minor congenital malformations were not in themselves severe enough to disrupt the atrioventricular conduction system. The cases fitted well into the categorization of complete heart block suggested by Lev. Two exhibited lack of communication between the atrial and conducting tissues, the other had discontinuity of the penetrating atrioventricular bundle. In an attempt to explain why this discontinuity between different segments of the conducting tissues occurs, we re-examined several series of graded human embryos. This investigation suggested that the anulus fibrosus in the normal heart is derived from sulcus tissue of the atrioventricular junction, the endocardial atrioventricular cushions playing a minor role in the separation of atria from ventricles. The relationships between the sulcus tissues and the different components of the atrioventricular junctional area are discussed in terms of an explanation both for the existence of different types of congenitally complete heart block and for persistence of Mahaim (nodo-ventricular and nodo-fascicular) fibers.
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