Abstract
Objective: Regular blood transfusions used for long term survival in ß-thalassemia major patients cause a secondary state of tissue iron overload. Myocardial iron deposition can result in cardiomyopathy, and heart failure remains the leading cause of death. This study was planned to see the correlation of Relative Wall Thickness (RWT), LV Mass, Cardiac Index(CI) and pulmonary capillary wedge pressure(PCWP) in relation to Serum Ferritin in transfusion dependent Beta Thalassemia children.
 Methods: Patients of ß thalassemia major above 2 years of age received regular blood transfusions at least for 1 year duration, attending OPD in the Department of Pediatrics, S.P. Medical College, Bikaner were enrolled. Echo findings of 50 cases were correlated with serum ferritin level(SFL).
 Result: Mean RWT in SFL group <2500ng/ml was 0.40±0.09, in SFL group 2500-5000 ng/ml, it was 0.41±0.08 and in SFL group >5000 ng/ml, it was 0.49±0.09; p value>0.05. Mean LV mass in SFL group <2500 was 68.76±24.32, in SFL group 2500-5000, it was 90.07±24.18 and in SFL group >5000, it was 123.06±42.42. The difference was found statistically highly significant (p<0.001). Mean PCWP in SFL group <2500 was 11.55±1.53, in SFL group 2500-5000, it was 12.02±2.06 and in SFL group >5000, mean PCWP was 13.31±2.09;p value>0.05. Mean CI in SFL group <2500 was 5.24±0.99, in 2500-5000 group was 5.79±1.07 and in SFL group >5000, it was 5.91±1.26 ; p value>0.05.
 Conclusion: There was significant positive correlation of serum ferritin level only with LV Mass.Relative Wall thickness, PCWP and cardiac index were insignificantly correlated. .
 Keywords: ß-thalassemia major; Relative wall thickness, LV mass/ LV mass index, cardiac index, PCWP, Echocardiogram; Tissue Doppler Imaging
Highlights
There are at least 60,000 individuals born with ßthalassemia major (TM) each year[1]
Eccentric hypertrophy shows normal periphery resistance but high cardiac index consist with excess circulating blood volume while concentric remodeling it characterized by high peripheral resistance, low cardiac index, and increase arterial stiffness In the present study, pulmonary capillary wedge pressure (PCWP) was calculated by using mitral E velocity to early diastolic mitral annulus velocity ( Em) ratio, with the formula[26]: pulmonary capillary w edge pressure (PCWP)=1.9+1.24(E/Em) In the present study, only 40% (n=20) of children were noted to have high PCWP
The present study showed that, out of total serum ferritin >2500ng/ml patients, 52% and 43% of the patients had LV mass and LV mass index >97th centile
Summary
There are at least 60,000 individuals born with ßthalassemia major (TM) each year[1]. The ongoing deaths from cardiac iron loading may relate to inadequate compliance or genetic factors related to metal transporters not yet fully elucidated[6,7,8], but whatever the cause, there is strong evidence that long-term deferoxamine chelation does not effectively prevent myocardial siderosis in a majority of patients[9,10].Deferiprone, the first approved oral chelator, has been shown in randomized controlled trials to be effective monotherapy at 100 mg/kg/day dose in treating mild to moderately severe myocardial iron loading (myocardial T2* 8–20 ms), significantly improving both myocardial iron and ejection fraction[11], and the combination of deferiprone at 75 mg/kg/day with deferoxamine is likewise effective[12]. Cardiac iron overload or the iron overload cardiomyopathy is regarded as the most serious condition and is the leading cause of morbidity and International Journal of Medical and Biomedical Studies (IJMBS)
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