Volume 68 Number 3 May 2001	back to contents

The Mount Sinai Hospital Clinicopathological Conference: A 45-Year-Old Man with Pompe's Disease and Dilated Cardiomyopathy	205-212
Ronen Ben-Ami, M.D., Jeffrey Puglisi, M.D., Tauseef Haider, M.D., and Davendra Mehta, M.D., Ph.D.
Address correspondence to Davendra Mehta, M.D., Ph.D., Director, Clinical Electrophysiology Laboratory, Box 1054, Mount Sinai School of Medicine, One East 100th Street, New York, NY. This is an unusual case of a 45-year-old man, born in Ecuador, with evidence of profound left ventricular dysfunction, dilated cardiomyopathy and marked myocardial hypertrophy. Preceding events were advanced atrioventricular block (necessitating pacemaker implantation) and atrial flutter. The diagnosis of Pompe's disease was established by endomyocardial biopsy and appropriate staining, which indicated abnormal glycogen storage. KEY WORDS Dilated cardiomyopathy, heart block, glycogen storage disease, Pompe's disease, Chagas' disease, congestive heart failure, ventricular dysfunction, Fabry's disease.

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