Wilson's Disease and Cardiac Myopathy

Grandis, Donald J.; Nah, Gregory; Whitman, Isaac R.; Vittinghoff, Eric; Dewland, Thomas A.; Olgin, Jeffrey E.; Marcus, Gregory M.

doi:10.1016/j.amjcard.2017.08.025

Cited by 45 publications

(37 citation statements)

References 11 publications

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“…Cardiac manifestations such as arrhythmias, autonomic dysfunction, cardiomyopathy, and sudden cardiac death have been found and previously reported in adult patients with Wilson's disease. [3][4][5][6] Kuan et al were the first to report electrocardiographic abnormalities, cardiomyopathy, and sudden cardiac death in 18 out of 53 Wilson's disease patients, and they suggested that cardiac involvement could be related to functional alterations in myocardial tissue due to low or high copper levels. 15 Cardiovascular autonomic dysfunction due to involvement of both parasympathetic and sympathetic systems was previously reported in adult Wilson's disease patients with neurological presentation.…”

Section: Discussionmentioning

confidence: 99%

“…2 Cardiac problems such as arrhythmias, autonomic dysfunction, cardiomyopathy, and sudden cardiac death occur in adult patients with Wilson's disease. [3][4][5][6] To date, cardiac issues in Wilson's disease have been the subject in some case reports and investigated in small cohorts, especially among adult patients. Therefore, cardiac involvement has not been reported in large longitudinal cohorts due to the lack of routine cardiac evaluation of these patients during their follow-up.…”

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confidence: 99%

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Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

et al. 2019

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Background:This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson’s disease.Method:Asymptomatic patients with a diagnosis of Wilson’s disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched.Results:The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias.Conclusion:Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

et al. 2019

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“…Cardiac arrhythmias, cardiomyopathy, and sudden circulatory death are rare complications but have been reported in children due to myocardial copper accumulation . Echocardiography is a valuable tool for surveillance of patients with WD who, as adults, are at increased risk of heart failure and atrial fibrillation . Therapy includes oral copper chelation therapy or LT for progressive liver failure, which may stabilize both cardiac and liver status.…”

Section: Inherited Metabolic Diseasementioning

confidence: 99%

Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

et al. 2019

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There is close interaction between the functions of the liver and heart affecting the presentation, diagnosis, and outcome of acute and chronic cardiac and liver disease. Conditions affecting both organ systems should be considered when proposing transplantation because the interaction between cardiac disease and liver disease has implications for diagnosis, management, selection for transplantation, and, ultimately, for longterm outcomes after liver transplantation (LT). The combination of cardiac and liver disease is well recognized in adults but is less appreciated in pediatric patients. The focus of this review is to describe conditions affecting both the liver and heart and how they affect selection and management of LT in the pediatric population.

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“…The disease is caused by mutations of the ATP7B gene on chromosome 13, which encodes for a P-type ATPase that is mainly expressed in the liver. 2,3 Here, we describe an unprecedented case of cardiac arrest in a patient with WD 1 year after curative liver transplantation. 1 The latter results in a continuous accumulation of copper in the liver, with subsequent cellular injury and tissue damage.…”

Section: Introductionmentioning

confidence: 97%

“…Rarely, accumulation of copper in the heart causes conduction abnormalities, arrhythmias, cardiomyopathy, and/or sudden cardiac death. 2,3 Here, we describe an unprecedented case of cardiac arrest in a patient with WD 1 year after curative liver transplantation.…”

Section: Introductionmentioning

confidence: 97%

Cardiac arrest in Wilson's disease after curative liver transplantation: a life‐threatening complication of myocardial copper excess?

et al. 2019

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We report the case of a 38‐year‐old man who presented with cardiac arrest 1 year after curative liver transplantation for Wilson's disease. Clinical work‐up proofed myocardial copper and iron accumulation using mass spectrometry, which led most likely to myocardial fibrosis as visualized by cardiovascular magnetic resonance (unprecedented delayed enhancement pattern) and endomyocardial biopsy. Consequently, cardiac arrest due to ventricular fibrillation and subsequent episodes of sustained ventricular tachycardia were considered as primary cardiac manifestation of Wilson's disease. This can, as illustrated by our case, occur even late after curative liver transplantation, which is an important fact that treating physicians should be aware of during clinical follow‐up of these patients.

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Wilson's Disease and Cardiac Myopathy

Cited by 45 publications

References 11 publications

Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

Cardiac arrest in Wilson's disease after curative liver transplantation: a life‐threatening complication of myocardial copper excess?

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