Evaluation of cardiac function in paediatric Wilson's disease patients with advanced echocardiographic modalities (strain and strain rate echocardiography).

Objective: In Wilson's disease (WD), copper accumulation in the organs and/or damage caused by oxygen free radicals occurs due to disturbances in copper excretion. In our study, we aimed to evaluate cardiac involvement with advanced echocardiographic modalities (tissue Doppler echocardiography, strain and strain-rate echocardiography).

Methods: Twenty WD patients and 20 healthy children from the Pediatric Gastroenterology Department of Diyarbakır Children's Hospital were included in the study between 2022 and 2023.

Results: The mean age of the WD patients was 12.89 ± 3.79 years. Left ventricular wall thicknesses and diameters (diastolic interventricular septum thickness, diastolic left ventricular posterior wall thickness, left ventricular end-diastolic diameter), left ventricular diastolic function parameters (E, A, E/A, deceleration time) and left ventricular ejection fraction and tricuspid annular plane systolic excursion were similar and not statistically significantly different in the WD and control groups. Mitral lateral e', mitral septal e' and tricuspid lateral e' velocities were lower in the WD patients and statistically significantly different from the controls (p = 0.02, 0.04 and 0.005, respectively), as assessed by tissue Doppler echocardiography. Global longitudinal systolic strain was similar in the WD and control groups and no statistically significant difference was detected. Longitudinal early diastolic strain rate was lower in the WD patients and statistically significantly different (p = 0.002).

Conclusions: Subclinical early diastolic dysfunction and segmental systolic dysfunction were detected in WD patients with advanced echocardiographic modalities, in addition to normal cardiac function as assessed by conventional echocardiography. Advanced echocardiographic modalities can be used in the follow up of WD patients.