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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 27, No 5, September/October 2016
314
AFRICA
(
p
<
0.05), mid-anterior and basal anterior segments, and
end-systolic longitudinal strain [SLSC (ES)] of the basal septal
and lateral segments, and the most negative peak longitudinal
strain (SLSC peak S) of the mid-anterior segment were
statistically significant lower in the patient group (
p
<
0.05).
Our study showed that in the early stages of Wilson’s disease,
diastolic dysfunction due to copper accumulation may be
heterogenous but in the long term, the heart could be affected
globally. Early diastolic dysfunction can be detected using strain
and strain rate echocardiography.
Conclusion
Cardiac arrhythmias, cardiomyopathy and sudden cardiac death
are rare complications but may be seen in children with Wilson’s
disease due to copper accumulation in the heart tissue. Strain
and strain rate echocardiography is a relatively new and useful
echocardiographic technique to evaluate cardiac function and
cardiac deformation abnormalities. In our study, despite normal
systolic function, the patients with Wilson’s disease showed
diastolic dysfunction and regional deformation abnormalities,
especially rotational strain and strain rate abnormalities. We
suggest that diastolic dysfunction, and rotational strain and
strain rate abnormalities in Wilson’s disease may be progressive
in untreated patients.
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