Cardiac abnormalities in children with thalassemia major: correlation of echocardiographic parameters with serum ferritin levels

Abstract

Myocardial iron deposition causes cardiac hypertrophy and dilatation and degeneration of myocardial fibres. The unbound iron generates toxic oxygen free radicals resulting in further injury to the myocytes. Patients develop conductive disturbances and progressive heart failure. 2 Regular monitoring for cardiac dysfunction and ABSTRACT Background: Thalassemias are a group of inherited disorders of haemoglobin synthesis, with significant morbidity and mortality. The leading cause of death in transfused thalassemia major children is myocardial haemosiderosis. Echocardiographic estimation of left ventricular function may reveal abnormalities before there is any clinical evidence of cardiac disease. The aim of the study was to determine the magnitude and nature of cardiac involvement in children with thalassemia major with respect to various serum ferritin levels. Methods: A prospective study was done on 30 cases of Thalassemia major attending our tertiary referral centre for a period of two yrs. The relationship of blood transfusion and serum ferritin with the parameters PWT-d, PWT-s, LViedd, LViesd, septal thickness, E/A and ES were studied. Results: Out of 30 patients studied, mean age was 4.02 yrs, mean number of transfusions was 28.97 and mean serum ferritin level was 1475. Mean serum ferritin levels of the patients receiving transfusions were estimated in various groups, ferritin levels were higher (2610 ±115.33) in patients who had received more than 50 transfusions.3 of our patients had features of left ventricular hypertrophy in ECG, rest were normal and none of them had evidence of cardiac arrhythmia. Echo parameters were compared; an increase in LViesd was noted in cases when compared to controls which was statistically significant (0.006). Conclusions: Persistent ferritin levels greater than 2500 mcg/ml and assessment of annual blood requirement can assist in prediction of development of cardiac disease. Thus, regular monitoring for cardiac dysfunction and arrhythmias with annual echocardiogram and ECG is extremely important.

Evaluation of Relationship between Serum Ferritin Level and Cardiac Complications in Thalassemia Patients in Pasteur Hospital of Bam City in 2018

“…Due to changes in the social, cultural and health structure of the affected area and the prevalence of family marriages in south and east of Kerman province and lack of knowledge of families about the consequences of family marriages, including the births of children with major thalassemia, it is necessary to examine the patients with thalassemia major in this area. Based on the results of this study, the mean age of the patients was 17.84 ± 6.72 years while in the study conducted by Niranjan et al, the mean age of the patients was 4.02 ± 3.37 years (16), which is not consistent with the results of our study. In the study conducted by Morad Veisi et al, the mean age of the subjects was 18.21 ± 8.05 (17), which is consistent with the findings of our study.…”

“…In the study conducted by Nouri et al, the mean heart rate of patients was 92 ± 16 (4). In the research conducted by Niranjan study, ECG was abnormal in only 3 (10%) patients (16). In the study conducted by Faruqi et al, sinus tachycardia was seen in 44.5% of patients (21), indicating that ECG alone is not sufficient to diagnose heart problems and complications in thalassemia patients.…”

“…In this center, the age of onset of blood transfusion was lower, but the blood transfusion intervals were almost the same. In the study conducted by Niranjan et al, blood transfusion intervals were 28.97 days (16). In the study conducted by Madmali et al, the mean transfusion interval was 29.79 days (29) and in the study conducted by Shamsyan, the mean transfusion interval was 24.2 days (30).…”

“…There was no statistically significant correlation between the age of onset of blood consumption and intervals of transfusions and cardiac complications and echocardiographic findings. In the study conducted by Niranjan, there was a correlation between the echocardiographic findings and the frequency of transfusions in patients undergoing transfusions under 50 (16). However, in the study conducted by Nathan DG, the echocardiographic findings were in individuals who had over 100 transfusions (24).…”

The correlation between serum ferritin levels and impaired heart function in children with transfusion dependent thalassemia

“…Data analysis used the Spearman correlation with p <0.05.Results: There were 45 children with a median age of 10 (2-17) years and had received a median of 18 (10-51) blood transfusions. The mean serum ferritin level was 4,321 (1,233) ng / mL. Blood transfusion frequency was associated with an increase in serum ferritin (rho 0.74, P <0.005).…”