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.
D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000150:complications / Q000175:diagnosis; D006332:Cardiomegaly / Q000150:complications / Q000175:diagnosis; D002648:Child; D004562:Electrocardiography; D000795:Fabry Disease / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male