CARDIAC PROBLEMS IN THE GLYCOGENOSES WITH SPECIFIC REFERENCE TO POMPE'S DISEASE

Publisher Website
Google Scholar
Abstract
In 4 of 16 patients having glycogenoses other than Pompe's disease electrocardiographic abnormalities were found. These abnormalities could not be related to the type of glycogenosis present and were limited to changes in the T wave and the QT interval. No other evidence of heart disease was found in these patients. Hemodynamic and cineangiographic evidence of muscular obstruction to cardiac outflow from both right and left ventricles was documented in a case of Pompe's disease. The obstruction was confirmed by postmortem findings. In one other case not investigated by cardiac catheterization, similar autopsy findings were found. This alteration is probably more widespread than is generally known, and has important therapeutic implications.