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Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma.

W P Follansbee, E I Curtiss, T A Medsger

    The New England Journal of Medicine
    |January 19, 1984
    PubMed
    Summary
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    Progressive systemic sclerosis with diffuse scleroderma often causes abnormal heart muscle blood flow (myocardial perfusion) due to microcirculation issues. This can lead to both right and left ventricular dysfunction, suggesting heart damage from poor blood supply.

    Area of Science:

    • Cardiology
    • Rheumatology
    • Pulmonology

    Background:

    • Progressive systemic sclerosis (PSS) with diffuse scleroderma can affect cardiopulmonary function.
    • Cardiac involvement is not always clinically evident in PSS patients.

    Purpose of the Study:

    • To investigate cardiopulmonary function in patients with PSS and diffuse scleroderma.
    • To identify the prevalence and nature of cardiac abnormalities in this patient group.

    Main Methods:

    • Studied 26 patients with PSS using maximal exercise thallium scans, radionuclide ventriculography, pulmonary-function testing, and chest roentgenography.
    • Assessed myocardial perfusion and ventricular function at rest and during exercise.

    Main Results:

    Related Experiment Videos

  • 20 out of 26 patients showed abnormal thallium scans, indicating widespread myocardial perfusion issues.
  • Abnormalities included reversible and fixed perfusion defects, suggesting microcirculatory disturbances.
  • Lower ejection fractions were observed in patients with higher scores for post-exercise left ventricular thallium defects, linking perfusion defects to ventricular dysfunction.

    • Conclusions:

    • Myocardial perfusion abnormalities are common in PSS with diffuse scleroderma, likely stemming from microcirculatory disturbances.
    • Both right and left ventricular dysfunction are associated with these circulatory issues, pointing towards ischemically mediated myocardial injury.