The heart in Wilson's disease
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Wilson's disease affects multiple organ systems including the cardiovascular system. Cardiovascular complications of Wilson's disease include cardiomyopathy, heart failure and cardiac arrhythmias.
Clinical Findings
A 10-year prospective study identified several patterns of cardiovascular involvement among 54 patients with Wilson’s disease.[1] Cardiovascular involvement was not present at the time of entry into the study:
Arrhythmias
More than 50% of patients developed electrocardiographic (ECG) abnormalities. These abnormalities included:
- Left ventricular hypertrophy
- Biventricular hypertrophy
- Early repolarization
- ST depression and T inversion
- Premature atrial contractions
- Premature ventricular contractions
- Atrial fibrillation
- Sinoatrial block
- Mobitz type 1 atrioventricular block
Tremor artifacts during ECG examinations in patients with Wilson's disease should also been considered (Resting tremors of the arms constitute the major problem in patients with Wilson's Disease).
- Cardiomyopathy; interstitial fibrosis and myocarditis,
- Cardiac Death; ventricular fibrillation and complications of cardiomyopathies
- Autonomic Dysfunction: abnormal response to the Valsalva maneuver
Histopathological Findings
According to a 9-patient histological study, the main characteristics are;
- Interstitial fibrosis,
- Sclerosis of the intramyocardial small vessels
- Perivascular myocarditis.
In autopsy results, myocardial copper concentrations in cardiac Wilson's disease patients went from low (2.28 μgm / g18) to high (1.428 μgm / g). The differing results could have arisen from varying responses to copper chelating therapy. The increased copper didn't seem to correspond to the severity of the myocardial lesions. [2]
References
- ↑ Kuan P (1987). "Cardiac Wilson's disease". Chest. 91 (4): 579–83. PMID 3829752. Unknown parameter
|month=ignored (help) - ↑ Factor SM, Cho S, Sternlieb I, Scheinberg IH, Goldfischer S (1982). "The cardiomyopathy of Wilson's disease. Myocardial alterations in nine cases". Virchows Arch A Pathol Anat Histol. 397 (3): 301–11. PMID 7157667.