Key result
Wilson's disease did not significantly alter left ventricular global longitudinal strain compared to controls (-21.8% vs -22.8%; p=0.124), though global radial and right ventricular strain were reduced.
Why the study?
Wilson's disease causes copper deposition into organs including the heart with structural changes seen even without heart failure, but CMR-based strain analysis had not been evaluated as a prognostic marker.
Does cardiac magnetic resonance-based strain analysis detect early myocardial functional impairment in patients with Wilson's disease compared to matched controls?
Observational (n=122)
Does cardiac magnetic resonance-based strain analysis detect early myocardial functional impairment in patients with Wilson's disease compared to matched controls?
Absolute Event Rate: -21.8% vs -22.8%
p-value: p=0.124
Cardiac magnetic resonance strain analysis can detect early subclinical functional impairment of the left and right ventricles in asymptomatic patients with Wilson's disease.
Early ventricular strain impairment may signal subclinical cardiac involvement in WD; leaves open need for routine CMR screening.
(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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Zhang et al. (2021) conducted an observational in Wilson's disease (n=122). Wilson's disease vs. Age and sex-matched controls was evaluated on Left ventricular global longitudinal strain (GLS) (p=0.124). Wilson's disease did not significantly alter left ventricular global longitudinal strain compared to controls (-21.8% vs -22.8%; p=0.124), though global radial and right ventricular strain were reduced.
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