Electrocardiographic and Echocardiographic Findings in Pre-Liver Transplant Pediatric and Young Adult Patients With Wilson’s Disease: A Case-Control Study

Document Type : Original Article


1 Neonatology and Cardiac Research Center, Shiraz University of Medical Science, Shiraz, Iran.

2 Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran

3 Gastroenterhepatology Research Center, Shiraz University of Medical Science, Shiraz, Iran.


Background: Wilson’s cardiac involvement causes cardiomyopathy, arrhythmia, autonomic nervous system dysfunction, and sudden cardiac death. This study aimed to evaluate cardiac dysfunction in pre-liver transplant patients suffering from Wilson’s disease and to classify their risk of arrhythmia and sudden cardiac death.
Methods: This case-control study was performed in the Transplant Coordination Center at Namazi Hospital, Shiraz University of Medical Sciences, between 2012 and 2014. The cardiac function was evaluated with 12-lead electrocardiography and echocardiography (M-mode, color Doppler, and tissue Doppler imaging). P-wave dispersion, QT dispersion, and T peak to T end-dispersion were measured in the patient group, and these values were compared with those in the control group.
Results: Totally, 23 patients with Wilson’s disease and 47 healthy individuals were included in this study. P-wave dispersion and QT dispersion were significantly increased in the patients with Wilson’s disease (P<0.05). Pulsed Doppler echocardiographic findings showed significantly increased E and A peak velocities of the mitral and tricuspid annuli in the patient group (P<0.05). Tissue Doppler imaging was in favor of a significant increase in systolic and early and late diastolic velocities of the mitral and tricuspid annuli.
Conclusions: The prolongation of P-wave dispersion and QT dispersion renders patients with Wilson’s disease susceptible to atrial and ventricular arrhythmias and sudden cardiac death. The evaluation of the cardiac function of such patients should include color Doppler and tissue Doppler imaging to assess diastolic dysfunction as one of the initial cardiac involvements. (Iranian Heart Journal 2022; 23(1): 118-128)


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