Abstract: Objective To analyze the manifestations of cardiac involvement in 60 patients with hepatolenticular degeneration. Methods Between January 2018 and December 2022, 60 patients with hepatolenticular degeneration were diagnosed and treated in Yellow River Sanmenxia Hospital. Clinical data of the patients were collected, and a 12 lead electrocardiogram was used for examination. Abnormal cases were examined using a color Doppler ultrasound diagnostic instrument for echocardiography, and serum cardiac markers were detected using chemiluminescence and double antibody sandwich immunofluorescence methods. Results 60 patients with hepatolenticular degeneration had liver manifestations as the first symptom, with alanine aminotransferase [(43.7 ± 6.8) U/L], aspartate aminotransferase [(46.5 ± 7.6) U/L], γ- Glutamic transferase [(65.7 ± 8.3) U/L], total bilirubin [(36.9 ± 4.1) μ mol/L] and 24-hour urine copper [(655.3 ± 140.9) μg/d] were significantly increased, while the levels of ceruloplasmin [(73.5 ± 9.2) mg/L] and serum copper [(5.8 ± 0.6) μ mol/L] significantly decreased. Among the 60 patients with hepatolenticular degeneration, 18 had cardiac involvement, with 4 cases of atrial premature beats and 4 cases of increased QRS complex wave width (22.2%), 2 cases of T-wave inversion, ST segment changes, and atrioventricular block (11.1%), and 1 case of ventricular tachycardia, P-wave inversion, sinus bradycardia, and coexistence of sinus bradycardia and T-wave inversion (5.6%). The left ventricular end diastolic diameter [(47.5 ± 7.6) mm], left ventricular ejection fraction [(69.2 ± 8.8)%], and E/A (1.9 ± 0.2) levels are relatively high. When the heart is affected in patients with hepatolenticular degeneration, serum levels of troponin I (cTnI) [(1.4 ± 0.1) ng/mL] and N-terminal B-type natriuretic peptide (NT proBNP) [(693.5 ± 162.7) pg/mL] significantly increase. Conclusion Heart involvement is more common in patients with Wilson's disease, with changes in cardiac structure and function that can be evaluated using imaging and laboratory examinations.

Key words: Hepatolenticular degeneration, Heart involvement, Electrocardiogram, Echocardiography, Heart biomarkers