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Related Experiment Videos

Lenegre's disease in youth

S B Dianzumba, D H Singer, S Meyers

    American Heart Journal
    |October 1, 1977
    PubMed
    Summary

    This case study details a young man with Lenegre's disease, experiencing progressive heart block. Early diagnosis and understanding of conduction abnormalities are crucial for managing this rare condition.

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    Area of Science:

    • Cardiology
    • Electrophysiology
    • Genetics

    Background:

    • Lenegre's disease is a rare, idiopathic conduction system disease primarily affecting the His-Purkinje system.
    • It typically presents in older adults, characterized by progressive atrioventricular (AV) block without structural heart disease.

    Observation:

    • A 22-year-old male presented with activity-related lightheadedness and fatigue, progressing to significant incapacity.
    • Electrocardiograms (ECGs) revealed intermittent complete trifascicular block, and Holter monitoring showed symptoms correlated with advanced AV block.
    • His bundle electrograms indicated infra-nodal block localized to the bundle branch system, with a normal A-H interval and prolonged H-Q interval.

    Findings:

    • The patient exhibited progressive bilateral bundle branch disease without other cardiac lesions or family history, meeting criteria for Lenegre's disease.
    • This case represents the youngest reported patient diagnosed with Lenegre's disease.
    • Electrophysiological studies pinpointed the block's location within the His-Purkinje system.

    Implications:

    • This case expands the known age spectrum for Lenegre's disease, highlighting its potential onset in young adulthood.
    • Understanding the electrophysiologic basis of block and exercise-induced conduction changes is vital for patient management.
    • Early identification and characterization of conduction system disease are essential for timely intervention and improved outcomes.

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