The heart in Wilson's disease: Difference between revisions
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==Overview== | |||
[[Wilson's disease]] affects multiple systems. Various medical conditions have been linked with the copper accumulation that accompanies Wilson's, including [[cardiomyopathy]] (weakness of the heart muscle), which is a rare but recognized problem; it may lead to [[heart failure]] (fluid accumulation due to decreased pump function) and [[cardiac arrhythmia]]s (episodes of irregular and/or abnormally fast or slow heart beat) | |||
==Clinical Findings== | |||
A 10-year, prospective study with 53 patients reported that there are four group of clinical symptoms in Wilson’s disease.<ref>Kuan P, Cardiac Wilson's disease Chest 1987; 91; 579-583 DOI 10.1378/chest.91.4.579 </ref> At the beginning of this study (at the time of presentation), cardiovascular abnormalities were not noted in any of these patients (all the patients in this report presented without any cardiac symptoms); | |||
*Arrhythmias: More than 50% of patients have ECG signs of left ventricular hypertrophy, biventricular hypertrophy, early repolarization, and ST depression and T inversion, premature atrial or 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’s 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. <ref> Factor SM, Cho S, Sternlieb I, Scheinberg IH, Goldfischer S. The cardiomyopathy of Wilsonıs disease: myocardial alteration in nine cases. Virchow’s Arch A Pathol Anat 1982; 397:301-11</ref> | |||
==References== | |||
{{reflist|2}} | |||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
Revision as of 17:47, 4 May 2009
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Overview
Wilson's disease affects multiple systems. Various medical conditions have been linked with the copper accumulation that accompanies Wilson's, including cardiomyopathy (weakness of the heart muscle), which is a rare but recognized problem; it may lead to heart failure (fluid accumulation due to decreased pump function) and cardiac arrhythmias (episodes of irregular and/or abnormally fast or slow heart beat)
Clinical Findings
A 10-year, prospective study with 53 patients reported that there are four group of clinical symptoms in Wilson’s disease.[1] At the beginning of this study (at the time of presentation), cardiovascular abnormalities were not noted in any of these patients (all the patients in this report presented without any cardiac symptoms);
- Arrhythmias: More than 50% of patients have ECG signs of left ventricular hypertrophy, biventricular hypertrophy, early repolarization, and ST depression and T inversion, premature atrial or 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’s 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]