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Systemic sclerosis associated with right ventricular cardiomyopathy

A P Bewley1, J P Cooper, N J Levell

  • 1Department of Dermatology, University College London Medical School, U.K.

The British Journal of Dermatology
|June 1, 1996
PubMed
Summary
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Systemic sclerosis can affect the heart, sometimes causing right ventricular cardiomyopathy even with normal pulmonary artery pressures. This case highlights a less common cardiac manifestation of this multisystem disorder.

Area of Science:

  • Cardiology
  • Rheumatology
  • Systemic Autoimmune Diseases

Background:

  • Systemic sclerosis is a rare multisystem disease of unknown cause, primarily affecting the skin.
  • Cardiac involvement is common in systemic sclerosis, often affecting the left ventricle.
  • Right ventricular dysfunction typically correlates with pulmonary vascular disease.

Observation:

  • This report details a patient with systemic sclerosis.
  • The patient presented with right ventricular cardiomyopathy.
  • Pulmonary artery pressures in this patient were within the normal range.

Findings:

  • Systemic sclerosis can lead to right ventricular cardiomyopathy.
  • Right ventricular cardiomyopathy may occur in systemic sclerosis independent of pulmonary hypertension.

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  • This presentation is atypical as right ventricular dysfunction usually implies pulmonary vascular disease.

    • Implications:

    • Recognizing cardiac involvement in systemic sclerosis is crucial for patient management.
    • Right ventricular cardiomyopathy should be considered in systemic sclerosis patients, even without elevated pulmonary pressures.
    • Further research is needed to understand the mechanisms of cardiac dysfunction in systemic sclerosis.