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.
Pathophysiology
Accumulation of copper in myocardial tisse is postulated to be the underlying mechansim of disease in the patient with Wilson's disease. At autopsy myocardial copper concentrations in patients with Wilson's disease ranged from (2.28 μgm / g) to (1.428 μgm / g). It has been speculated that the wide range in copper concentrations could have arisen from varying responses to copper chelation therapy. Increased copper levels were not correlated with the severity of the myocardial lesions rasing questions as to the role of the patient's autoimmune response to the copper deposition. Extracellular and not intracellular copper levels were evaluated. [1]
Clinical Findings
A 10-year prospective study identified several patterns of cardiovascular involvement among 54 patients with Wilson’s disease.[2] 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
Wilson's Disease is often associated with resting tremors. Therefore, before diagnosing atrial fibrillation or other ECG abnormalities, tremor artifacts on the ECG should also be excluded in the patient with Wilson's disease.
Cardiomyopathy
On biopsy and or pathologic evaluation the following abnormalities can be present:
- Interstitial fibrosis
- Sclerosis of the intramyocardial small vessels
- Perivascular myocarditis
Autonomic Dysfunction
As a manifestation of autonomic dysfunction, there can be an abnormal response to the Valsalva maneuver.
Complications from Cardiovascular Involvement
In some patients with Wilson's disease ventricular fibrillation and complications of progressive heart failure due to a cardiomyopathy can result in death.
References
- ↑ 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.
- ↑ Kuan P (1987). "Cardiac Wilson's disease". Chest. 91 (4): 579–83. PMID 3829752. Unknown parameter
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