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Voluntary muscle involvement in hypertrophic cardiomyopathy. A study of eleven patients

E R Smith, L P Heffernan, V E Sangalang

    Annals of Internal Medicine
    |November 1, 1976
    PubMed

    Insights

    Hypertrophic cardiomyopathy involves more than just heart muscle. This study found muscle abnormalities in patients, suggesting a wider disease spectrum affecting both cardiac and voluntary muscles.

    Area of Science:

    • Cardiology
    • Neurology
    • Muscle Physiology

    Background:

    • Hypertrophic cardiomyopathy (HCM) is typically viewed as a primary cardiac muscle disease.
    • Clinical evidence suggests a potentially broader pathological involvement beyond the heart.

    Purpose of the Study:

    • To investigate the extent of muscle involvement in patients diagnosed with hypertrophic cardiomyopathy.
    • To determine if abnormalities exist in voluntary muscles of individuals with HCM.

    Main Methods:

    • Electromyography (EMG) was performed on 11 patients with hypertrophic cardiomyopathy.
    • Voluntary muscle biopsies were obtained and analyzed using light and electron microscopy.

    Main Results:

    • EMG revealed myopathic findings in 10 out of 10 patients, including reduced potential amplitude and duration.
    • Muscle biopsies showed abnormalities in 8 out of 11 patients, such as central core/target fibers and mitochondrial proliferation.
    • Subsarcolemmal mitochondrial proliferation was observed in six patients, with some exhibiting abnormal ultrastructure.

    Conclusions:

    • Hypertrophic cardiomyopathy is associated with widespread muscle abnormalities, extending beyond cardiac muscle.
    • The findings suggest HCM is part of a larger disease spectrum affecting both cardiac and skeletal muscles.
    • This indicates a systemic myopathic process in patients with hypertrophic cardiomyopathy.

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