Kun Zhang1,2,3, Ulrike Reuner4, Charlotte Hempel5, Uwe Speiser5, Karim Ibrahim6, Frank R Heinzel1,3, Burkert Pieske1,2,3, Marian Christoph6, Felix M Heidrich5, Silvio Quick6. 1. Department of Internal Medicine and Cardiology, Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, 13353 Berlin, Germany. 2. Berlin Institute of Health (BIH), 10178 Berlin, Germany. 3. DZHK (German Centre for Cardiovascular Research), Partner Site Berlin, 10785 Berlin, Germany. 4. Department of Neurology, Technische Universität Dresden, University Hospital, 01307 Dresden, Germany. 5. Department of Internal Medicine and Cardiology, Technische Universität Dresden, Herzzentrum Dresden Universitätsklinik, 01307 Dresden, Germany. 6. Department of Cardiology, Technische Universität Dresden, Klinikum Chemnitz gGmbH, 09116 Chemnitz, Germany.
Abstract
(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.
(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 WDpatients, 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. WDpatients -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). WDpatients had significantly reduced global radial strain (p = 0.002). Right ventricular GLS was also significantly reduced in WDpatients (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 WDpatients.
Entities:
Keywords:
Wilson’s disease; cardiac magnetic resonance imaging; feature-tracking; global longitudinal strain; strain analysis
Authors: Z Hlubocká; Z Marecek; A Linhart; E Kejková; L Pospísilová; P Martásek; M Aschermann Journal: J Inherit Metab Dis Date: 2002-08 Impact factor: 4.982
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