The heart in Wilson's disease: Difference between revisions
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==Cardiomyopathy== | ==Cardiomyopathy== | ||
On biopsy and or | On biopsy and or pathologic evaluation the following abnormalities can be present: | ||
*Interstitial [[fibrosis]] | *Interstitial [[fibrosis]] | ||
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*Perivascular [[myocarditis]] | *Perivascular [[myocarditis]] | ||
==Autonomic Dysfunction== | |||
As a manifestation of autonomic dysfunction, there can be an abnormal response to the [[Valsalva maneuver]]. | |||
==Complications from Cardiovascular Involvement== | ==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== | ==References== |
Revision as of 11:46, 5 May 2009
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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
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
- ↑ Kuan P (1987). "Cardiac Wilson's disease". Chest. 91 (4): 579–83. PMID 3829752. Unknown parameter
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