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Accelerated atrioventricular conduction in Fabry's disease: a case report

J W Rowe, D G Caralis

    Angiology
    |July 1, 1978
    PubMed

    Insights

    Fabry's disease can lead to accelerated atrioventricular conduction, a heart rhythm disorder. This may be due to abnormal His bundle potentials caused by glycolipid deposition in affected individuals.

    Area of Science:

    • Cardiology
    • Genetics
    • Biochemistry

    Background:

    • Fabry's disease (angiokeratoma corporis diffusum universale) is a rare genetic disorder.
    • It results from lysosomal alpha-galactosidase A deficiency, leading to glycolipid accumulation.
    • Cardiac involvement, including conduction abnormalities, is a known complication.

    Observation:

    • Three unrelated male patients with Fabry's disease and accelerated atrioventricular conduction were studied.
    • One patient exhibited a prolonged deflection on His bundle electrogram during tachycardia episodes.
    • Progressive PR interval shortening was observed in two patients over 10-13 years.

    Findings:

    • Accelerated atrioventricular conduction is a potential complication of Fabry's disease.
    • Abnormal His bundle potentials, possibly due to glycolipid deposition, may underlie these conduction issues.
    • The observed PR interval changes suggest a progressive cardiac involvement.

    Implications:

    • Early recognition of cardiac conduction abnormalities is crucial in managing Fabry's disease.
    • This finding highlights the importance of cardiac monitoring in patients with Fabry's disease.
    • Understanding the mechanism of conduction defects can inform therapeutic strategies for Fabry's disease.

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