Secondary athrocytotic cardiomyopathy ? Heart damage due to Wilson's disease

Abstract

Post-mortem atomic absorption spectrophotometry of the myocardium of a 14-year-old boy revealed a hundred-fold increase in copper. Further electrolyte analysis of the myocardium showed changes corresponding to metabolic heart muscle damage. Ultrastructural examination showed all the feature of a cardiomyopathy at the cellular level. Laser-Microprobe-Mass-Analysis demonstrated an inhomogeneous distribution of copper. An essential factor in the mechanism of death is heart damage.

P wave dispersion is prolonged in patients with Wilson’s disease

“…A recent report Kaduk et al [27] describing cardiomyopathy in Wilson's disease, suggested that mitochondrial alterations were the consequence of the accumulation of myocardial copper These alterations are non-specific, and in some cases of limited severity; however, in previous study it was concluded that cardiac degeneration might have contributed to the death of Wilson's patients [27] . PWD may well be associated with autonomic dysfunction in patients with Wilson's disease [27] . Dysautonomia, often subclinical, is only one of the many features of Wilson's disease [28] .…”

Myocardial integrated ultrasonic backscatter for early detection of cardiac involvement in patients with Wilson disease

“…Morphologic abnormalities and myocardial alterations consistent with cardiomyopathy have been previously reported in autopsy specimens of the hearts of Wilson disease patients (30). Myocardial damage and the presence of myocardial copper deposition 10-100 times the normal concentration have also been reported in the autopsy tissues of Wilson disease patients (4,39). However, conflicting results on the clinical impact of myocardial copper levels have been reported in previous studies (1,39,40).…”

“…Myocardial damage and the presence of myocardial copper deposition 10-100 times the normal concentration have also been reported in the autopsy tissues of Wilson disease patients (4,39). However, conflicting results on the clinical impact of myocardial copper levels have been reported in previous studies (1,39,40). A limitation of our study is that we have no data on the copper levels in regional myocardial tissues and magnetic resonance imaging was not performed.…”

“…A study by Kaduk et al (39) on cardiomyopathy in Wilson disease patients suggested that mitochondrial alterations are the consequence of myocardial copper accumulation. These alterations are also nonspecific and present in some cases of limited severity; however, a previous study concluded that cardiac degeneration might contribute to death in Wilson disease patients (39).…”

“…Cardiac manifestations in Wilson disease patients include many ECG abnormalities and arrhythmias such as atrial fibrillation, early repolarization, ST depression, T inversion, sinoatrial block, Mobitz type I AV block, tremor artifact (1,3,4), supraventricular tachycardia and frequent supraventricular ectopic beats (36), post-transplant electrical storm (37), P-wave dispersion abnormalities (38), cardiomyopathy (3,29,30,39,40), orthostatic hypotension and autonomic dysfunction (1,41), and cardiac deaths due to ventricular fibrillation and cardiac failure (29). Morphologic abnormalities and myocardial alterations consistent with cardiomyopathy have been previously reported in autopsy specimens of the hearts of Wilson disease patients (30).…”