Dataset Information

The impact of Wilson disease on myocardial tissue and function: a cardiovascular magnetic resonance study.

ABSTRACT:

Background

Systemic effects of altered serum copper processing in Wilson Disease (WD) might induce myocardial copper deposition and consequently myocardial dysfunction and structural remodeling. This study sought to investigate the prevalence, manifestation and predictors of myocardial tissue abnormalities in WD patients.

Methods

We prospectively enrolled WD patients and an age-matched group of healthy individuals. We applied cardiovascular magnetic resonance (CMR) to analyze myocardial function, strain, and tissue characteristics. A subgroup analysis of WD patients with predominant neurological (WD-neuro⁺) or hepatic manifestation only (WD-neuro^-) was performed.

Results

Seventy-six patients (37 years (27-49), 47% women) with known WD and 76 age-matched healthy control subjects were studied. The prevalence of atrial fibrillation in WD patients was 5% and the prevalence of symptomatic heart failure was 2.6%. Compared to healthy controls, patients with WD had a reduced left ventricular global circumferential strain (LV-GCS), and also showed abnormalities consistent with global and regional myocardial fibrosis. WD-neuro⁺ patients presented with more severe structural remodeling and functional impairment when compared to WD-neuro^- patients.

Conclusions

In a large cohort, WD was not linked to a distinct cardiac phenotype except CMR indexes of myocardial fibrosis. More research is warranted to assess the prognostic implications of these findings.

Trial registration

This trial is registered at the local institutional ethics committee (S-188/2018).

SUBMITTER: Salatzki J

PROVIDER: S-EPMC8223377 | biostudies-literature | 2021 Jun

REPOSITORIES: biostudies-literature

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Publications

The impact of Wilson disease on myocardial tissue and function: a cardiovascular magnetic resonance study.

Salatzki Janek J Mohr Isabelle I Heins Jannick J Cerci Mert H MH Ochs Andreas A Paul Oliver O Riffel Johannes J André Florian F Hirschberg Kristóf K Müller-Hennessen Matthias M Giannitsis Evangelos E Friedrich Matthias G MG Merle Uta U Weiss Karl Heinz KH Katus Hugo A HA Ochs Marco M

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance 20210624 1

<h4>Background</h4>Systemic effects of altered serum copper processing in Wilson Disease (WD) might induce myocardial copper deposition and consequently myocardial dysfunction and structural remodeling. This study sought to investigate the prevalence, manifestation and predictors of myocardial tissue abnormalities in WD patients.<h4>Methods</h4>We prospectively enrolled WD patients and an age-matched group of healthy individuals. We applied cardiovascular magnetic resonance (CMR) to analyze myoc ...[more]

PMID: 34162411

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Dataset Information

The impact of Wilson disease on myocardial tissue and function: a cardiovascular magnetic resonance study.

Background

Methods

Results

Conclusions

Trial registration

Publications

The impact of Wilson disease on myocardial tissue and function: a cardiovascular magnetic resonance study.

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