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The conduction system in Pompe's disease.

S Bharati, M Serratto, I DuBrow

    Pediatric Cardiology
    |January 1, 1982
    PubMed
    Summary
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    Infants with glycogen storage disease show microscopic changes in the heart conduction system. These changes, including enlarged cells and altered topography, may explain rapid electrical conduction and short P-R intervals on ECG.

    Area of Science:

    • Cardiology
    • Pediatric Pathology
    • Electrophysiology

    Background:

    • Glycogen storage diseases (GSDs) can affect multiple organs, including the heart.
    • Cardiac conduction abnormalities have been anecdotally reported in GSDs.
    • Microscopic details of the cardiac conduction system in GSDs are not well-characterized.

    Purpose of the Study:

    • To investigate the microscopic anatomy of the cardiac conduction system in infants with GSD.
    • To correlate electrophysiologic findings with structural changes in the conduction system.
    • To explore potential mechanisms for observed electrophysiologic abnormalities in GSD.

    Main Methods:

    • Microscopic examination of the cardiac conduction system in four infants with GSD.
    • Detailed electrophysiologic studies in one infant, including P-A, A-H, and H-V interval measurements.

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  • Histopathological analysis focusing on cell size, glycogen content, and topographical arrangement of conduction tissues.

    • Main Results:

    • Electrophysiologic studies revealed normal P-A and A-H intervals, with a slightly prolonged H-V interval.
    • Microscopic findings included enlarged cardiac cells, likely due to increased glycogen.
    • The topography of the atrioventricular conduction system was altered, with bulging of the ventricular septum summit, possibly due to cellular enlargement.

    Conclusions:

    • The observed rapid conduction in GSD may not be localized to the H-V interval's anatomic correlate.
    • Enlarged cardiac cells and altered conduction system topography in GSD are significant findings.
    • These structural changes may contribute to the short P-R interval seen on ECG in GSD patients.