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Idiopathic hypertrophic subaortic stenosis with split His bundle potentials. Electrophysiologic and pathologic

S Bharati, J H McAnulty, M Lev

    Circulation
    |December 1, 1980
    PubMed

    Insights

    Idiopathic hypertrophic subaortic stenosis can cause conduction system abnormalities. This case highlights the potential link between these findings and sudden cardiac death in young patients.

    Area of Science:

    • Cardiology
    • Electrophysiology
    • Cardiac Pathology

    Background:

    • Idiopathic hypertrophic subaortic stenosis (IHSS) is a significant cause of left ventricular outflow tract obstruction.
    • Conduction system abnormalities are common in IHSS but their direct link to sudden cardiac death requires further elucidation.

    Observation:

    • A 25-year-old female with IHSS presented with left bundle branch block.
    • Electrophysiologic studies revealed a prolonged His-bundle (HB) interval and split His potentials.
    • Histological examination showed fibrotic changes in the His-Purkinje system, particularly disruption of the left bundle branch.

    Findings:

    • There was a strong correlation between the patient's electrocardiographic and electrophysiologic findings and the autopsy results of the conduction system.
    • The prolonged HB interval and disrupted His-Purkinje system were significant findings in this patient with IHSS.

    Implications:

    • This case underscores the potential for severe conduction system disease in IHSS.
    • The findings suggest that conduction abnormalities may contribute to the risk of sudden cardiac death in patients with IHSS, possibly due to arrhythmias or outflow obstruction.

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