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    QTc nterval is prolonged in Wilson's disease with neurologic nvolvement
    (Taylor & Francis Ltd, 2018) Ozturk, Semi; Gurbuz, Ahmet Seyfeddin; Efe, Suleyman Cagan; Iliaz, Raim; Banzragch, Mutse; Demir, Kadir
    Background Neurologic and liver involvement in Wilson's disease (WD) is well-documented, however, few reports demonstrated cardiac involvement. Tpe and Tpe/QT are new measures of ventricular repolarization which were recently suggested as predictor of arrythmogenesis. We aimed to evaluate ventricular depolarization and repolarization parameters including QT, QTc, Tpe intervals, Tpe/QT, Tpe/QTc ratios, and QT dispersion (QTd) in patients with WD.Materials and methods Thirty-five patients with WD and 30 healthy controls were included in the study. Patients were evaluated by a neurologist in addition to MR imaging. Twenty-one of 35 patients were diagnosed as neuroWilson (NW), whereas 14 patients as non-NW. ECG recordings were obtained using a 12-lead commercial device (Cardiac Science, Burdick s500,USA). All patients underwent standard echocardiographic evaluation. These two groups of patients and healthy controls were compared.Results There were no difference between patients with WD and healthy controls in terms of age sex, BMI, liver, and kidney functions where as patients with WD were anemic and thrombocytopenic. Left atrial, ventricular dimensions, left ventricular systolic, and diastolic functions were similar between patients and healthy control. QT interval was prolonged in patient group, however, QTc, Tpe intervals, Tpe/QT, and Tpe/QTc ratios and QTd did not differ between groups. When patients with NW and non-NW were compared, both QT and QTc intervals were significantly longer in patients with NW, however, Tpe interval, Tpe/QT and Tpe/QTc ratios, and QTd did not differ.Conclusion QT and QTc intervals are prolonged in patients with Wilson's disease and neurologic involvement.
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    Strain and strain rate echocardiography variables in adult Wilson's disease patients: A speckle tracking echocardiography study
    (Wiley, 2020) Cagan, Suleyman; Gurbuz, Ahmet Seyfettin; Ozturk, Semi; Demir, Kadir
    Purpose Although the hepatic and neurological consequences of Wilson's disease (WD) have been investigated in detail, its cardiac involvement remains little studied. Our aim was to investigate potential cardiac differences in strain (ST) and strain rate (STR) echocardiography in adult WD patients compared with controls. Methods We included 30 patients with WD and a control group of 26 sex and age matched healthy adults. None of the subjects in either group had cardiac complaint. WD patients were clinically evaluated by a neurologist and undergone cranial magnetic resonance imaging. They were then divided into two groups according to the presence (NW) or absence (non-NW) of neurological involvement. Standard and advanced speckle tracking echocardiographic evaluations were performed in each group according to guidelines. Results Left ventricular (LV) systolic and diastolic diameters and wall thickness measurements were within normal limits and did not differ significantly between the groups (P > .05). Neither atrial peak longitudinal and circumferential ST variables nor LV global and longitudinal ST and STR variables showed significant differences between the NW, the non-NW, and the control group (P > .05). Conclusion Our echocardiographic study showed no detectable difference between adult WD patients with or without neurological involvement and healthy subjects.