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Electrocardiograms in Fabry's disease.

K J Sheth, J P Thomas

    Journal of Electrocardiology
    |April 1, 1982
    PubMed
    Summary
    This summary is machine-generated.

    Fabry's disease, a genetic disorder, can cause cardiac abnormalities like left ventricular hypertrophy (LVH) and conduction issues, even in younger individuals. Early ECG screening is crucial for detecting these heart problems in affected families.

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    Area of Science:

    • Cardiology
    • Genetics
    • Biochemistry

    Background:

    • Fabry's disease is a rare genetic disorder affecting the alpha-galactosidase A enzyme.
    • Cardiac involvement is a significant complication of Fabry's disease.
    • Electrocardiography (ECG) can reveal cardiac abnormalities.

    Purpose of the Study:

    • To investigate the prevalence and types of ECG abnormalities in individuals with Fabry's disease.
    • To assess cardiac involvement in affected males and heterozygous females.
    • To correlate ECG findings with enzyme levels and disease status.

    Main Methods:

    • Studied ECGs of 47 kindred members from three families with Fabry's disease.
    • Included a control group of 19 individuals with normal enzyme levels.

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  • Analyzed ECG parameters such as left ventricular hypertrophy (LVH) and conduction times.

    • Main Results:

    • Affected males (12) frequently showed ECG evidence of LVH (10/12) and ST-T wave changes (5/12).
    • One affected male exhibited accelerated cardiac impulse conduction.
    • Heterozygous females (16) did not show LVH but presented conduction abnormalities, including decreased PR index (4/16) and delayed conduction (2/16).

    Conclusions:

    • Fabry's disease is associated with significant ECG abnormalities, including LVH and conduction disturbances.
    • Both affected males and heterozygous females can exhibit cardiac involvement detectable by ECG.
    • ECG screening is valuable for identifying cardiac manifestations in Fabry's disease patients and carriers.