Myocardial integrated ultrasonic backscatter for early detection of cardiac involvement in patients with Wilson disease

Abstract

Background/Aims: Videodensitometry is a feasible noninvasive ultrasound tissue characterization method allowing early detection of myocardial changes. This study aimed to investigate ultrasonic backscatter properties of the myocardium in Wilson disease patients. Materials and Methods: We compared cardiologically asymptomatic Wilson disease patients (W group) (n=18) with age-matched (26.7±9.6 years) healthy controls (C group) (n=15). Diagnosis of Wilson disease was made on the basis of clinical manifestations, family history, and laboratory findings and confirmed by liver biopsy. Transthoracic echocardiographic quantitative texture analysis was performed on data from the septum and left ventricular posterior wall, and mean gray level (MGL) histograms at end-diastole (d) and end-systole (s) were obtained after background correction (c). Cyclic variation index (CVI) was calculated using the formula [(cMGLd -cMGLs) / cMGLd] ×100. Results: There were no significant differences in sex, age, body mass index, heart rate or blood pressure, and conventional echocardiographic parameters between the 2 groups. The cMGLs value of the posterior wall was higher in the W group than in the C group (30.9±2.6 vs. 22.2±2.7, p=0.033). The W group had a significantly lower CVI of the septum than did the C group (-22±4.4% vs. 43.4 ±12.9%, p<0.001), and there was no significant difference in the CVI of the posterior wall (-67.0±15.9% vs. 41.7±18.6%, p=0.32). Conclusion: Abnormalities in two-dimensional echocardiographic grey-level distributions were present in Wilson disease patients. These videodensitometric myocardial alterations were significantly lower in Wilson disease patients than in the controls, and this probably represents an early stage of cardiac involvement.

Autonomic Dysfunction in Wilson's Disease: A Comprehensive Evaluation during a 3-Year Follow Up

“…BRS is a sensitive predictor for worse prognosis in many chronic diseases, especially in heart diseases (La Rovere et al, 1998 ; Pinna et al, 2005 ). Cardiac abnormality is a common finding in Wilson's disease (Factor et al, 1982 ; Hlubocka et al, 2002 ; Arat et al, 2014 ). Factor et al revealed cardiac hypertrophy, interstitial and replacement fibrosis, intramyocardial small vessel sclerosis and focal inflammation in an autopsy study of Wilson's disease, though these changes were not significantly correlated with the tissue levels of copper or the presence of cirrhosis.…”

Cardiac assessment in Wilson’s disease patients based on electrocardiography and echocardiography examination

“…Arat et al studied 18 patients with WD and found normal LV size and function, and the ECG showed only an increase in the P wave dispersion [37]. In another study from the same group of researchers, the abnormalities in two-dimensional echocardiographic grey-level distributions in patients with WD were described as a result of an early stage of cardiac involvement [38]. In a recently published study Karakurt et al reported that pediatric patients with WD showed diastolic dysfunction and regional deformation abnormalities, especially rotational strain and strain rate abnormalities [39].…”

Cardiac arrhythmia in Wilson’s disease: An oversighted and overlooked entity!

“…Recently, myocardial integrated ultrasonic backscatter has been reported to detect early changes in myocardium of patients with Wilson's disease. [7]…”