Evaluation of ventricular arrhythmia in children with Wilson's disease; cardiac electrophysiological balance index (iCEB)

Abstract

Aim: To evaluate cardiac involvement in Wilson's disease (WD) noninvasively by electrocardiography and to analyze it with the cardiac electrophysiological balance index (iCEB). Method: Eighteen Wilson patients and 18 healthy child patients who were followed up in the Pediatric Gastroenterology department between 2022-2023 were included in the study. Results: Wilson disease patients had normal ventricular and autonomic functions. QT-dispersion (QT-d) (22.61 (±11.47), p=0.000) and Tpe (66.50 (40-78), p=0.02) were found to be significantly higher in the WD group. QRS, QRS-dispersion (QRS-d), QT, QTc, Tpe/QT ratio, Tpe/QTc ratio, QT/QRS ratio, QTc/QRS ratio, Tpe/QRS ratio, Tpe/(QT*QRS) ratio both had similar values in the groups. Heart rate variability parameters (SDNN, SDNN-i, SDANN, rMSSD, pNN50, LF/HF ratio) were at similar values in both groups. rMSSD, pNN50, which indicates parasympathetic activity, was lower in Wilson patients than in the control group, but no statistical difference was detected. LF/HF ratio was significantly higher in WD patients. Conclusions: Despite normal ventricular function and autonomic function in WD patients, they have an increased risk of ventricular arrhythmia. Although the cardiac electrophysiological balance index (iCEB) can provide useful information in the follow-up of WD patients, we recommend that depolarization, repolarization times, and repolarization dispersion times be evaluated separately in addition to iCEB.