Electrocardiograms in Fabry's disease.

We studied the ECGs of 47 kindred members from three families with Fabry's disease. The control group (19) with normal plasma alpha-galactosidase levels had normal ECGs. Of the 12 affected males (mean age 15 years, plasma alpha-galactosidase levels less than 10% of normals), ten had ECG evidence of LVH, and five had ST-T wave changes. The conduction time of the cardiac impulse was accelerated (decreased PR index) in one male. Of the 16 heterozygous females (mean age 19 years, plasma alpha-galactosidase level and alpha/beta galactosidase ratio in the carrier range) none had ECG evidence of LVH, but six had conduction abnormalities--decreased PR index (four) and delayed conduction (two). The ECG abnormalities were uniformly distributed in different age groups.