Cardiac and autonomic function in patients with Wilson’s disease

Quick, Silvio; Reuner, Ulrike; Weidauer, Marie; Hempel, Charlotte; Heidrich, Felix M.; Mues, Christoph; Sveric, Krunoslav Michael; Ibrahim, Karim; Reichmann, Heinz; Linke, Axel; Speiser, Uwe

doi:10.1186/s13023-019-1007-7

Cited by 15 publications

(25 citation statements)

References 28 publications

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“…However, structural changes are rarely seen in the usually stable WD patients under medical treatment, when transferred to standard cardiac examination [ 4 ]. As presented previously, asymptomatic WD patients had grossly normal systolic left heart (ejection fraction, end diastolic diameter) and right heart function (end diastolic diameter, tricuspid annular plane systolic excursion, right ventricular systolic pressure) in echocardiography; only mild diastolic dysfunction was present compared to healthy controls [ 9 ].…”

Section: Discussionmentioning

confidence: 90%

“…Patients were part of the CARMA Wilson’s disease study (Cardiac manifestation of Wilson’s disease). Conventional CMR data, echocardiography and autonomic function data were published elsewhere [ 9 , 10 ].…”

Section: Methodsmentioning

confidence: 99%

“…Recently, we described that signs for structural changes are even present in asymptomatic WD patients who still have normal left and right ventricular dimensions and systolic function in echocardiographic assessment. WD was associated with a higher incidence of premature ventricular ectopic beats (PVB) and severity of WD based on the Unified Wilson’s Disease Rating Scale was significantly correlated to NT-pro BNP [ 9 ]. Cardiac magnetic resonance imaging (CMR) unmasked an increase in late gadolinium enhancement (LGE) and a decrease in the right ventricular ejection fraction in WD patients compared to the control [ 10 ].…”

Section: Introductionmentioning

confidence: 99%

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Evaluation of Myocardial Strain Using Cardiac Magnetic Resonance in Patients with Wilson’s Disease

Zhang

Reuner

Hempel

et al. 2021

JCM

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(1) Background: Wilson’s disease (WD) is an inherited autosomal recessive disorder with the excessive deposition of copper into different organs, including the heart. Previous studies showed structural cardiac changes even in patients with no signs of heart failure. The aim of this study was to perform cardiac magnetic resonance-based strain analysis in WD patients, as it is a powerful independent predictor of mortality. (2) Methods: We conducted a prospective cardiac magnetic resonance study that included 61 patients and 61 age and sex-matched controls, and performed strain analysis of the left and right ventricle. (3) Results: Left ventricular global longitudinal strain (GLS) as a prognostic marker of increased mortality was not altered (control −22.8 (4.8) % vs. WD patients −21.8 (5.1) %, p = 0.124). However, 4 of the 61 patients had a markedly reduced GLS. Global circumferential strain did not significantly differ between the groups either (p = 0.534). WD patients had significantly reduced global radial strain (p = 0.002). Right ventricular GLS was also significantly reduced in WD patients (p = 0.01). (4) Conclusions: Strain analysis revealed functional impairment of the left and right ventricle in a small number of patients as a potential early sign of cardiac manifestation in asymptomatic WD patients.

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Section: Discussionmentioning

confidence: 90%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Evaluation of Myocardial Strain Using Cardiac Magnetic Resonance in Patients with Wilson’s Disease

Zhang

Reuner

Hempel

et al. 2021

JCM

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“…However, cases of cardiac sudden death and an increased incidence of arrhythmias and heart failure have been reported. Just recently, we have demonstrated that even asymptomatic patients exhibit structural disease and autonomic dysfunction of the heart [1, 2].…”

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Left ventricular clefts – incidental finding or pathologic sign of Wilson’s disease?

Zhang

Reuner

Weidauer

et al. 2019

Orphanet J Rare Dis

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BackgroundWilson’s disease is an inherited autosomal recessive multi-systemic disorder characterized by reduced excretion and consequently excessive accumulation of copper in different organs, such as the heart.ResultsIn a prospective controlled trial, which is the largest to date, we evaluated 61 patients with Wilson’s disease, age- and sex-matched to 61 healthy patients, for cardiac manifestation using cardiac magnetic resonance imaging. Patients were under stable disease and had no signs of heart failure at the time of examination.We detected a left ventricular cleft, an invagination penetrating more than 50% wall thickness of the adjoining compact myocardium in diastole, in 20% of the patients (12 out of 61) compared to 5% among control patients (3 out of 61, p = 0.013). No correlation between the incidence of cleft and a certain genotype of Wilson’s disease was found. All described cases were incidental findings and none of the patients showed other signs of cardiac involvement.ConclusionsTo conclude, the results of this study suggests that the increased occurrence of left ventricular clefts is due to Wilson’s disease. Large studies with a long observation period are needed for further evaluation.

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“…Elkiran et al studied 22 children with Wilson's disease, and theyreported diastolic dysfunction[18]. In another study, the LV systolic function measured by ejection fraction did not differ significantly between Wilson's disease and controls, however, diastolic dysfunction was more frequent in patients with Wilson's disease[19].Hlubocka Z et al compared 2D echocardiographic data of 42 patients withWilson's disease to 42 healthy subjects. They reported a non-significant trend towards lower LV ejection fraction values in patients with Wilson's disease (62% versus 64%), the LV diastolic function did not differ in the two groups[20].In a recent study by Quick et al, 61 patients with Wilson's disease were evaluated by cardiac magnetic resonance.…”

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Assessment of LV Function in Children with Wilson’s Disease: Speckle Tracking Imaging Study

Ahmed¹,

Yahia²,

Yaseen³

et al. 2019

WJCD

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Background and Objective: Wilson's disease is a genetic disorder of copper metabolism that affects liver and other organs including heart. In early stages of myocardial affection, the left ventricle (LV) appears apparently normal when evaluated by traditional two-dimensional (2D) echocardiography. The aim of this study was to detect subclinical LV dysfunction in children with Wilson's disease using 2D speckle tracking echocardiography. Patients and Methods: Twenty children with Wilson's disease were compared with ageand sex-matched 20 healthy children. All subjects were evaluated by traditional 2D echocardiography and speckle tracking echocardiography. Results: There were no significant differences between patients and controls regarding conventional echo parameters except for lower E mitral flow and E' annular septal peak velocity in patient group. The regional peak longitudinal strain of apical 4 chamber view was −17.8% ± 4.2% in patients and −20.1% ± 2.3 % in control subjects (P = 0.043), and for apical 2 chambers view, it was −20.1% ± 3.6% in patients and −22.6% ± 3.4% in control subjects (P = 0.034) and it was −18% ± 3.5% in patients and −20.5% ± 3.2% in control subjects (P = 0.025) in apical long axis view. The global peak longitudinal strain was also lower in patients than control group (18.3% ± 3.2%, and 20.85% ± 2.4%) respectively (P = 0.014). There were no significant differences between both groups regarding circumferential and radial strains (P > 0.05). Conclusions: Despite apparently normal LV systolic function, the children with Wilson's disease demonstrated significantly lower peak longitudinal strain as an indicator for early affection of LV systolic function.

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Cardiac and autonomic function in patients with Wilson’s disease

Cited by 15 publications

References 28 publications

Evaluation of Myocardial Strain Using Cardiac Magnetic Resonance in Patients with Wilson’s Disease

Evaluation of Myocardial Strain Using Cardiac Magnetic Resonance in Patients with Wilson’s Disease

Left ventricular clefts – incidental finding or pathologic sign of Wilson’s disease?

Assessment of LV Function in Children with Wilson’s Disease: Speckle Tracking Imaging Study

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