OBJECTIVE: Major thalassemia’s main therapy is blood transfusion, thus put it at
risk of iron overload, which is mostly affecting the heart. Iron chelating agent can’t
significantly prevent cardiac dysfunction caused by iron overload. The objective of
this study is to evaluate cardiac dysfunction in major thalassemia by assessing
echocardiography profile and its correlation with ferritin serum in children with major
thalassemia.
METHOD: A retrospective study was done in 45 thalassemia major children less
than 18 years old attending cardiology outpatient clinic in Dr. Soetomo Academic
General Hospital from January to December 2018, who underwent more than 10
period of transfusion. Ferritin serum level was taken from mean levels of 3
measurements, prior to echocardiography. Echocardiography examination was done
by two pediatric cardiologists using 3D Doppler device, evaluating the ejection
fraction (EF), tricuspid annular plane systolic excursion (TAPSE) and E/A ratio by
calculating early diastole (E wave) and atrial contarction (A wave). Spearman
correlation analysis was used with P value <0.05 considered as significant.
RESULT: About 25/45 subjects were boys, with median age 10 (2-17) years. Median
blood transfusions rate was 18 (10-51)times, while median serum ferritin level was
4,321 (1,168-15,233)ng/mL. The frequency of blood transfusion was correlated with
the enhancement of ferritin serum (r=0.74; P<0.001). Echocardiography examination
revealed 3/45 children had dilated cardiomyopathy. The mean value of EF was
68.69(SD 10.163)%, TAPSE 2.09 (SD 0.575)cm and E/A ratio 1.68 (SD 0.464). The
ferritin serum had negative correlation with ejection fraction (r=-0.78; P<0.001) and
TAPSE (r=-0.65; P<0.001), but had positive correlation with E/A ratio (r=0.67;
P<0.001).
CONCLUSION: Echocardiography examination of EF, TAPSE and E/A ratio can
describe the decreasing of heart function in children with major thalassemia