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

Zhang, Kun; Reuner, Ulrike; Hempel, Charlotte; Speiser, Uwe; Ibrahim, Karim; Heinzel, Frank R.; Pieske, Burkert; Christoph, Marian; Heidrich, Felix M.; Quick, Silvio

doi:10.3390/jcm10020335

Cited by 5 publications

(4 citation statements)

References 29 publications

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“…In our study, no significant difference was observed in 2D LV-Strain (GRS, GCS, GLS) between the WD and control groups, which was inconsistent with previous studies [6,8]. In the studies of Salatzki et al [6] and Zhang et al [8], LV-GRS and LV-GCS in WD patients were significantly lower than those in controls. This difference may be due to the younger patients in our study and the shorter duration of copper exposure.…”

Section: Discussioncontrasting

confidence: 99%

“…ECG abnormalities were found in 19 patients, including premature ventricular contractions (n = 4), sinus bradycardia (n = 11), and sinus arrhythmia (n = 4). The median UWDRS score of WD-neuro + patients was 6.0 (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11).…”

Section: Baseline Characteristicsmentioning

confidence: 97%

“…arrhythmias, atrial fibrillation, heart failure, etc.) [6][7][8][9][10][11][12][13]. Therefore, early detection of myocardial involvement is important even in asymptomatic WD patients.…”

Section: Introductionmentioning

confidence: 99%

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Myocardial involvement characteristics by cardiac MR imaging in neurological and non-neurological Wilson disease patients

Deng,

Zhang,

Jia

et al. 2024

Insights Imaging

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Objectives To explore the characteristics of myocardial involvement in Wilson Disease (WD) patients by cardiac magnetic resonance (CMR). Methods We prospectively included WD patients and age- and sex-matched healthy population. We applied CMR to analyze cardiac function, strain, T1 maps, T2 maps, extracellular volume fraction (ECV) maps, and LGE images. Subgroup analyzes were performed for patients with WD with predominantly neurologic manifestations (WD‐neuro +) or only hepatic manifestations (WD‐neuro −). Results Forty-one WD patients (age 27.9 ± 8.0 years) and 40 healthy controls (age 25.4 ± 2.9 years) were included in this study. Compared to controls, the T1, T2, and ECV values were significantly increased in the WD group (T1 1085.1 ± 39.1 vs. 1046.5 ± 33.1 ms, T2 54.2 ± 3.3 ms vs. 51.5 ± 2.6 ms, ECV 31.8 ± 3.6% vs. 24.3 ± 3.7%) (all p < 0.001). LGE analysis revealed that LGE in WD patients was predominantly localized to the right ventricular insertion point and interventricular septum. Furthermore, the WD‐neuro + group showed more severe myocardial damage compared to WD‐neuro − group. The Unified Wilson Disease Rating Scale score was significantly correlated with ECV (Pearson’s r = 0.64, p < 0.001). Conclusions CMR could detect early myocardial involvement in WD patients without overt cardiac function dysfunction. Furthermore, characteristics of myocardial involvement were different between WD‐neuro + and WD‐neuro − , and myocardial involvement might be more severe in WD‐neuro + patients. Critical relevance statement Cardiac magnetic resonance enables early detection of myocardial involvement in Wilson disease patients, contributing to the understanding of distinct myocardial characteristics in different subgroups and potentially aiding in the assessment of disease severity. Key points • CMR detects WD myocardial involvement with increased T1, T2, ECV. • WD‐neuro + patients show more severe myocardial damage and correlation with ECV. • Differences of myocardial characteristics exist between WD‐neuro + and WD‐neuro − patients. Graphical Abstract

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Section: Discussioncontrasting

confidence: 99%

Section: Baseline Characteristicsmentioning

confidence: 97%

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Myocardial involvement characteristics by cardiac MR imaging in neurological and non-neurological Wilson disease patients

Deng,

Zhang,

Jia

et al. 2024

Insights Imaging

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“…Therefore, in the diagnosis and the follow-up of WD patients, tests such as electrocardiogram, cardiac biomarkers, echocardiography, and 24-h Holter monitoring should be included [27]. By cardiac magnetic resonance-based strain analysis, a functional impairment of both ventricles was detected in 4 out of 61 WD patients, which may be helpful as a potential early sign of cardiac manifestation [28].…”

Section: Additional Clinical Manifestationsmentioning

confidence: 99%

Wilson’s Disease: Facing the Challenge of Diagnosing a Rare Disease

et al. 2021

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Wilson disease (WD) is a rare disorder caused by mutations in ATP7B, which leads to the defective biliary excretion of copper. The subsequent gradual accumulation of copper in different organs produces an extremely variable clinical picture, which comprises hepatic, neurological psychiatric, ophthalmological, and other disturbances. WD has a specific treatment, so that early diagnosis is crucial to avoid disease progression and its devastating consequences. The clinical diagnosis is based on the Leipzig score, which considers clinical, histological, biochemical, and genetic data. However, even patients with an initial WD diagnosis based on a high Leipzig score may harbor other conditions that mimic the WD’s phenotype (Wilson-like). Many patients are diagnosed using current available methods, but others remain in an uncertain area because of bordering ceruloplasmin levels, inconclusive genetic findings and unclear phenotypes. Currently, the available biomarkers for WD are ceruloplasmin and copper in the liver or in 24 h urine, but they are not solid enough. Therefore, the characterization of biomarkers that allow us to anticipate the evolution of the disease and the monitoring of new drugs is essential to improve its diagnosis and prognosis.

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Liver transplantation for Wilson disease: Current knowledge and future perspectives

Ferrarese,

Cazzagon,

Burra

2024

Liver Transplantation

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Liver transplantation currently represents a therapeutic option for patients Wilson’s disease presenting with end-stage liver disease or acute liver failure. Indeed, it has been associated with excellent post-operative survival curves, in view of young age at transplant and absence of recurrence. Attention has shifted over the past decades to a wise expansion of indications for liver transplantation. Evidence has emerged supporting transplantation of carefully selected patients with primarily neuropsychiatric symptoms and compensated cirrhosis. The rationale behind this approach is the potential for surgery to improve copper homeostasis and consequently ameliorate neuropsychiatric symptoms. However, several questions remain unanswered, such as how to establish thresholds for assessing pre-transplant neuropsychiatric impairment, how to standardize preoperative neurological assessments, and how to define post-operative outcomes for patients meeting these specific criteria. Furthermore, a disease-specific approach will be proposed both for the liver transplant evaluation of Wilson’s disease candidates and for patient care during the transplant waiting period, highlighting peculiarities of this systemic disease.

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

Cited by 5 publications

References 29 publications

Myocardial involvement characteristics by cardiac MR imaging in neurological and non-neurological Wilson disease patients

Myocardial involvement characteristics by cardiac MR imaging in neurological and non-neurological Wilson disease patients

Wilson’s Disease: Facing the Challenge of Diagnosing a Rare Disease

Liver transplantation for Wilson disease: Current knowledge and future perspectives

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