Subclinical Myocardial Dysfunction in Children With Wilson's Disease

Condition(s) studied

Investigational drug(s) / intervention(s)

Echocardiography: a safe, painless test that uses sound waves to create moving pictures of your heart's structure and pumping function.

Electrocardiography: a quick, painless test that records the electrical signals in the heart.

N-terminal pro b- type natriuretic peptide: a protein made by our heart, examined by peripheral blood sample.

Study summary

Wilson's disease (WD) is one of the most common metabolic liver diseases in older children. The most frequent clinical presentation is liver disease. However, Wilson's disease (WD) is a multisystem disorder. It is concluded that four modes of cardiac manifestations in Wilson's disease (WD) include arrhythmias, cardiomyopathy, cardiac death, and autonomic dysfunction. Such possible cardiac involvement should be added to the clinical picture of Wilson's disease (WD) involving the hepatic and central nervous system(CNS).

The data on cardiac manifestations in children is very limited and only few adult studies are available.

In this study, the investigators aim to unveil subclinical cardiac dysfunction in children with Wilson's disease with apparently normal cardiac functions by conventional assessment.

Eligibility

Primary outcome measure(s)

• Left Ventricular Peak Longitudinal Strain (LV-PLS) — Baseline (Day 1 , at single cross-sectional evaluation).
  Left Ventricular Peak Longitudinal Strain (LV-PLS) assessed by Speckle Tracking Echocardiography (STE) to evaluate subclinical LV systolic dysfunction. Expressed as a negative percentage (%), where a less negative percentage indicates impaired function.

Trial sites (2)

Other trials for the same condition