Electrocardiographic and vectorcardiographic abnormalities in Fabry's disease

Jawahar Mehta, Naip Tuna, James H. Moller, Robert J. Desnick

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68 Scopus citations


Fabry's disease has been reported to be associated with ECG abnormalities. Thirty-two patients with this disease followed in the University of Minnesota had ECG's and 15 had VCG's. An abnormal rhythm was observed in two patients on initial examination and four more developed abnormal rhythm on follow-up examinations. A short PR interval (120 msec. or less) was seen in five patients. Thirteen others had a PR interval that was less than 140 msec. Conduction abnormalities involving the A-V node or His bundle or its branches were present in 22 per cent of the patients, most frequently the intraventricular conduction defects progressing to the right bundle branch block. Atrial or ventricular enlargement was seen in 60 per cent of the patients, left ventricular hypertrophy being the most common. ST-T changes with or without chamber enlargement were seen in 10 patients. One patient had an anterior myocardial infarction pattern on his ECG. Hemizygosity was found to be associated with significantly more abnormalities than heterozygosity. The severity of conduction defects also increased with the duration of the disease process. Vectorcardiography in this study did not provide significant additional information other than that observed on the ECG alone. Since the pathology usually reveals myocardial fibers, conduction system, and blood vessels infiltrated with glycosphingolipid, it is believed that lipid infiltration is responsible for conduction defects, chamber enlargement, and other abnormalities. Although Fabry's disease is rare, it may be amenable to therapy; therefore, recognition of cardiac involvement is important.

Original languageEnglish (US)
Pages (from-to)699-705
Number of pages7
JournalAmerican Heart Journal
Issue number6
StatePublished - Jun 1977
Externally publishedYes

Bibliographical note

Funding Information:
From the Departments of Medicine, Pediatrics, and the Dight Institute for Human Genetics, University of Minnesota Medical School, Minneapolis, Minn. 55455. This work was supported in part by a grant (74-915) from the American Heart Association; a grant (1-273) from the National Foundation-March of Dimes; a grant (AM 15174) from the National Institutes of Health; and a grant (RR-400) from the General Clinical Research Center, Program of the Division of Research Resources, National Institutes of Health; Dr. Desnick is a recipient of a National Institutes of Health Career Development Award (1K04-AM00042). Received for publication Jan. 27, 1976. Reprint requests: J. Mehta, M.D., Box J-277, JHM Health Center, University of Florida, Gainsville, Fla. 32610.


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