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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
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[Hypertrophic non-obstructive cardiomyopathy (HNCM)--clinical, hemodynamic, electro-, echo- and angiocardiographic

H Kuhn, U Thelen, E Köhler

    Zeitschrift Fur Kardiologie
    |July 1, 1980
    PubMed

    Insights

    Hypertrophic non obstructive cardiomyopathy (HNCM) is a distinct clinical condition characterized by specific ECG and echocardiogram findings. This study defines HNCM, suggesting it may be more common than previously thought.

    Area of Science:

    • Cardiology
    • Internal Medicine
    • Genetics

    Context:

    • Hypertrophic non obstructive cardiomyopathy (HNCM) presents a diagnostic challenge.
    • Characterizing HNCM requires a comprehensive analysis of clinical, imaging, and hemodynamic data.

    Purpose:

    • To define the clinical features of hypertrophic non obstructive cardiomyopathy (HNCM).
    • To analyze ECG, echocardiogram, angiogram, hemodynamic, clinical course, and familial incidence in 49 HNCM patients.

    Summary:

    • ECG T-wave abnormalities (V4-V5) showed 91% specificity for HNCM.
    • Echocardiography revealed left ventricular apical obliteration (94%) and hypertrophy (72% asymmetric septal).
    • No storage disease found; 59% had exercise-induced pulmonary hypertension; 68% stable course over 5.1 years.

    Impact:

    • HNCM is a well-defined clinical entity.
    • This research suggests HNCM may be more prevalent than previously estimated.
    • Findings aid in better diagnosis and understanding of HNCM's clinical course and familial aspects.

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