The conduction system in Pompe's disease.
We report our findings in the microscopic examination of the conduction system in four infants with glycogen storage disease, one of whom had adequate electrophysiologic studies. The electrophysiologic studies in the latter case showed P-A and A-H intervals at the lower limits of normal, but the H-V interval was just above the normal mean. This suggests that the rapid conduction was not localized in the anatomic counterpart of the H-V interval. The short P-R interval in the ECG may be related to the enlargement of cells, which may in turn be related to increased glycogen content. The relationship of glycogen per se to the speed of conduction is unknown. We found that the summit of the ventricular septum bulged, probably because of the generally increased cell size, and that the topography of the atrioventricular conducting system was different from normal. This is possibly related both to an increase in the cell sizes of the specialized conducting tissue itself and to deforming effects of this bulging summit of the ventricular septum. New microscopic details of the components of the conducting system are described in these cases.
D006332:Cardiomegaly / Q000473:pathology; D004562:Electrocardiography; D005260:Female; D006008:Glycogen Storage Disease / Q000473:pathology*; D006009:Glycogen Storage Disease Type II / Q000473:pathology* / Q000503:physiopathology; D006329:Heart Conduction System / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D009336:Necrosis