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Related Experiment Videos

Cardiac involvement in Wilson disease.

Z Hlubocká1, Z Marecek, A Linhart

  • 1Second Department of Internal and Cardiovascular Medicine, First Faculty of Medicine, Charles University, Prague, Czech Republic.

Journal of Inherited Metabolic Disease
|September 14, 2002
PubMed
Summary

Cardiac involvement in Wilson disease (WD) is mild, showing increased left ventricular (LV) wall thickness and remodeling. Benign supraventricular tachycardias and extrasystolic beats are common in WD patients.

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Area of Science:

  • Cardiology
  • Genetics
  • Metabolic Disorders

Background:

  • Wilson disease is an inherited disorder of copper metabolism causing copper accumulation.
  • Cardiac involvement is a known, though less common, manifestation of Wilson disease.

Purpose of the Study:

  • To investigate cardiac involvement in patients with Wilson disease.
  • To assess cardiac structure and function using echocardiography and ECG Holter monitoring.

Main Methods:

  • Echocardiographic examination of 42 Wilson disease patients and 42 controls.
  • 24-hour ECG Holter monitoring in 23 Wilson disease patients.

Main Results:

  • Wilson disease patients showed increased interventricular septum and LV posterior wall thickness.

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  • Relative LV wall thickness was significantly increased, with 21% exhibiting concentric LV remodeling.
  • ECG monitoring revealed abnormalities in 42% of patients, mainly supraventricular tachycardias.

    • Conclusions:

    • Cardiac involvement in Wilson disease is generally mild.
    • Characterized by LV parietal thickening and increased concentric LV remodeling.
    • A high frequency of benign supraventricular tachycardias and extrasystolic beats was observed.