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The straight back syndrome

M K Davies, P Mackintosh, R M Cayton

    The Quarterly Journal of Medicine
    |January 1, 1980
    PubMed
    Summary
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    Straight back syndrome, a spinal condition, is linked to mitral valve prolapse, not pseudoheart disease. This inherited condition requires cardiac investigation and screening of relatives.

    Area of Science:

    • Cardiology
    • Genetics
    • Orthopedics

    Background:

    • Straight back syndrome (SBS) involves loss of thoracic spinal curvature, often mistaken for heart disease due to chest compression.
    • Previous understanding attributed SBS symptoms to cardiac compression, labeling it 'pseudoheart disease'.

    Purpose of the Study:

    • To investigate the association between straight back syndrome and cardiac abnormalities, particularly mitral valve prolapse.
    • To determine the inheritance pattern and diagnostic criteria for straight back syndrome.

    Main Methods:

    • Evaluated 31 patients with SBS and 45 relatives, performing clinical examinations and echocardiography.
    • Utilized lateral chest radiography to measure thoracic dimensions and HLA typing for genetic analysis.

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    Main Results:

    • A reduced distance between T8 and a T4-T12 line (less than 1.2 cm) indicated SBS.
    • 67% of subjects with SBS showed evidence of mitral valve prolapse.
    • SBS is inherited as an autosomal dominant condition, potentially linked to chromosome 6.

    Conclusions:

    • Straight back syndrome is not 'pseudoheart disease' but is associated with mitral valve prolapse.
    • Patients with SBS require cardiac evaluation, and family members should be screened for the condition.