Bradycardia-dependent block and fatigue phenomenon of the His-Purkinje system manifested during upright tilt.

Atrioventricular (AV) block occurring in the His-Purkinje system may occur sporadically and can be difficult to document. In this article, we describe two patients with spontaneous episodes of AV block, in whom the use of upright tilt during electrophysiological testing led to a diagnosis of His-Purkinje disease. In both cases, testing in the supine position only uncovered mild or no abnormalities of infra-nodal conduction. In the first case, high grade block distal to the His occurred and the mechanism appeared to be bradycardia-dependent block. In this case, increased sympathetic tone due to upright tilt may have enhanced the slope of phase 4 depolarization in abnormal His-Purkinje cells, leading to block. In the second case, high grade block distal to the His was seen with upright tilt and the mechanism of block appeared to be fatigue phenomenon in the His-Purkinje system. These cases emphasize the elusive nature and varied mechanisms of His-Purkinje block and illustrate the utility of electrophysiological testing in the upright position in patients with suspected conduction system disease.