Glycogen-storage disease of the heart: Hemodynamic and angiocardiographic features in 2 cases

In 2 cases of glycogen-storage disease of the heart the clinical features which distinguished this condition from other forms of “endomyocardial disease” were: (1) lack of skeletal muscular tone and atrophy of subcutaneous fat, (2) electrocardiographic findings of huge QRS complexes and a short P-R interval, (3) the familial tendency (1 case), and (4) positive skeletal muscular biopsies for glycogen storage disease. The angiocardiographic studies proved to be useful in evaluating the pathologic anatomy and hemodynamic status in each patient. In neither case were signs of subaortic stenosis observed by these studies. In the one case subjected to hemodynamic pressure studies, features of subaortic stenosis were not encountered.