Fifteen-year follow-up of a heterozygous Fabry's disease patient associated with pre-excitation syndrome

A 47-year-old woman with heterozygous Fabry's disease with pre-excitation s yndrome has been followed up for 15 years. Diagnosis was confirmed by the t ypical electron microscopic feature of the endomyocardial specimen and a de creased plasma a-galactosidase activity. As the disease progressed, the int erventricular septum thickened from 11 to 17 mm as measured by echocardiogr aphy, while the AH interval was prolonged from 80 to 140 msec, in Fabry's d isease, the PR interval has been reported to be variable from short PR to A V block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease.