Cardiovascular Journal of Africa: Vol 27 No 5 (September/October 2016)

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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 27, No 5, September/October 2016 AFRICA 307
Strain and strain rate echocardiography in children with
Wilson’s disease
Cemşit Karakurt, Serkan Çelik, Ayşe Selimoğlu, İlknur Varol, Hamza Karabiber, Saim Yolo ğlu
Abstract
Objective: This study aimed to evaluate strain and strain rate
echocardiography in children with Wilson’s disease to detect
early cardiac dysfunction.
Methods: In this study, 21 patients with Wilson’s disease and
a control group of 20 age- and gender-matched healthy chil-
dren were included. All the patients and the control group
were evaluated with two-dimensional (2D) and colour-coded
conventional transthoracic echocardiography by the same
paediatric cardiologist using the same echocardiography
machine (Vivid E9, GE Healthcare, Norway) in standard
precordial positions, according to the American Society of
Echocardiography recommendations. 2D strain and strain
rate echocardiography were performed after the ECG probes
of the echocardiography machine were adjusted for ECG
monitoring. Longitudinal, transverse and radial strain, and
strain rate were assessed from six basal and six mid-ventric-
ular segments of the left ventricle, as recommended by the
American Society of Echocardiography.
Results: Left ventricular wall thickness, systolic and dias-
tolic diameters, left ventricular diameters normalised to body
surface area, end-systolic and end-diastolic volumes, cardiac
output and cardiac index values were within normal limits
and statistically similar in the patient and control groups (p
> 0.05).
Global strain and strain rate: the patient group had a statisti-
cally significant lower peak A longitudinal velocity of the
left basal point and peak E longitudinal velocity of the left
basal (VAbasR) point, and higher global peak A longitudinal/
circumferential strain rate (GSRa) compared to the corre-
sponding values of the control group (p < 0.05).
Radial strain and strain rate: end-systolic rotation [ROT
(ES)] was statistically significantly lower in the patient group
( p < 0.05). Longitudinal strain and strain rate: end-systolic
longitudinal strain [SLSC (ES)] and positive peak transverse
strain (STSR peak P) were statistically significantly lower in
the patient group (p < 0.05).
Segmental analysis showed that rotational strain
measurement of the anterior and lateral segments of the
patient group were statistically significantly lower than
the corresponding values of the control group (p < 0.05).
Segmental analysis showed statistically significantly lower
values of end-systolic longitudinal strain [STSR (ES)] of the
basal lateral ( p < 0.05) and end-systolic longitudinal strain
[SLSC (ES)] of the basal septal segment ( p < 0.05) in the
patient group.
End-systolic longitudinal strain [SLSC (ES)] and posi-
tive peak transverse strain (STSR peak P) were statistically
significantly lower in the patient group (p < 0.05). Segmental
analysis showed statistically significantly lower values of end-
systolic longitudinal strain [SLSC (ES)] of the mid-anterior
and basal anterior segments (p < 0.05), end-systolic longitu-
dinal strain [STSR (ES)] measurements of the posterior and
mid-posterior segments, end-systolic longitudinal displace-
ment [DLDC (ES)] of the basal posterior, mid-posterior and
mid-antero-septal segments in the patient group.
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 abnormal-
ities. Our study showed that despite normal systolic function,
patients with Wilson’s disease showed diastolic dysfunction
and regional deformation abnormalities, especially rotational
strain and strain rate abnormalities.
Keywords: 2D strain, strain rate echocardiography, speckle track-
ing, Wilson’s disease
Submitted 14/7/14, accepted 8/3/16
Published online 13/5/16
Cardiovasc J Afr 2016; 27: 307–314
DOI: 10.5830/CVJA-2016-028
Wilson’s disease is an autosomal recessive metabolic liver disease
related to mutation of the copper-transporting ATPase, ATP7B,
an intracellular copper transporter mainly expressed in the
hepatocytes.1,2 Wilson’s disease is characterised by excessive
copper deposition in the body, primarily in the liver and brain,
resulting from inability of the liver to excrete copper in the bile.
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. 3-6 The
aims of our study were to determine potential differences in
strain and strain rate between patients with Wilson’s disease and
age-matched controls, and to detect early cardiac dysfunction.
Department of Pediatric Cardiology, Faculty of Medicine,
Inonu University, Malatya, Turkey
Cemşit Karakurt, MD,
Serkan Çelik, MD
Department of Pediatric Gastroenterology, Faculty of
Medicine, Inonu University, Malatya, Turkey
Ayşe Selimoğlu, MD
İlknur Varol, MD
Hamza Karabiber, MD
Department of Biostatistics, Faculty of Medicine, Inonu
University, Malatya, Turkey
Saim Yoloğlu, MD