Tissue Doppler echocardiography reliably reflects severity of iron overload in pediatric patients with β thalassemia

Silvilairat, Suchaya; Sittiwangkul, Rekwan; Pongprot, Yupada; Charoenkwan, Pimlak; Phornphutkul, Charlie

doi:10.1016/j.euje.2007.06.003

Cited by 28 publications

(32 citation statements)

References 28 publications

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“…β-TM patients with mean serum ferritin ≥2500 µg/l had significantly lower EF and higher TRV than those with serum ferritin <2500 µg/l ( Table 2). In agreement with our findings, Silvilairat et al [27] reported a preserved systolic and diastolic LV function in patients who have serum ferritin <2500 ng/ml. Hamdy and colleagues [55] showed that the presence of right ventricle systolic dysfunction in patients with β-TM is related to high levels of serum ferritin.…”

Section: Discussionsupporting

confidence: 82%

“…According to the literature, the cutoff value 2500 µg/l was used to classify patients into two groups as it was defined to be the best for prediction of thalassemia complication [26] and has been used for prognosis with higher incidence of cardiac complication and shortened survival in patients with levels >2500 µg/l compared with lower levels [27,28].…”

Section: Laboratory Analysismentioning

confidence: 99%

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Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

et al. 2017

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Background: Ischemia-modified albumin (IMA) is an altered type of serum albumin that forms under conditions of oxidative stress and an independent predictor of major adverse cardiovascular events. Objectives: To measure the levels of IMA in 45 children and adolescents with β-thalassemia major (β-TM) compared with 30 healthy controls and assess its relation to lipid peroxidation, vascular complications and subclinical atherosclerosis. Methods: β-TM patients without symptoms of heart disease were studied focusing on transfusion history, chelation therapy, serum ferritin, malondialdehyde (MDA) and IMA levels. Echocardiography was performed and carotid intima media thickness (CIMT) was assessed. Results: IMA and MDA levels were significantly higher in β-TM patients compared with controls (p < 0.001). IMA was higher among patients with heart disease, pulmonary hypertension risk and serum ferritin ≥2500 µg/l than those without. TM patients compliant to chelation had significantly lower IMA levels. IMA levels were positively correlated to MDA and CIMT while negatively correlated to ejection fraction and fractional shortening. Conclusion: Our results highlight the role of oxidative stress in the pathophysiology of vascular complications in thalassemia. IMA could be useful for screening of β-TM patients at risk of cardiopulmonary complications and atherosclerosis because its alteration occurs in early subclinical disease.

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Section: Discussionsupporting

confidence: 82%

Section: Laboratory Analysismentioning

confidence: 99%

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

et al. 2017

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“…In literature, we found few studies conducted in beta thalassemia patients which can be compared with our study (18)(19)(20)(21)(22). Specifically, tissue Doppler examination in the study performed by Vogel et al (18) included segmentary measurements as in our study.…”

Section: In 2001mentioning

confidence: 91%

“…When conventional findings of heart failure are observed on echocardiography, the prognosis of cardiomyopathy is frequently fatal (17). Recently, introduction of tissue Doppler for evaluation of diastolic function indicates that significant advances will be made in terms of early diagnosis of cardiomyopathy in patients with thalassemia major (18)(19)(20)(21)(22). In additon, usage of T2-star imaging method showing cardiac iron load has been the most important development for early diagnosis of risk of heart-related complications in recent years (23).…”

Section: Discussionmentioning

confidence: 99%

Beta talasemi majorlu çocuk ve ergenlerde doku Doppler yöntemiyle diyastolik işlevlerin değerlendirilmesi

Yavuz¹,

Nişli²,

Ertuğrul³

et al. 2011

tpa

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A As ss se es ss sm me en nt t o of f d di ia as st to ol li ic c f fu un nc ct ti io on n i in n c ch hi il ld dr re en n a an nd d a ad do ol le es sc ce en nt ts s w wi it th h b be et ta a--t th ha al la as ss se em mi ia a m ma aj jo or r b by y t ti is ss su ue e D Do op pp pl le er r i im ma ag gi in ng g T Ta an ne er r Y Ya av vu uz z, , K Ke em ma al l N Ni ifl fll li i* *, , N Na ac ci i Ö Ön ne er r* ** *, , T Tü ür rk ka an n E Er rt tu u¤ ¤r ru ul l* *, , Z Za af fe er r fi fia al lc c› ›o o¤ ¤l lu u * ** ** *, , G Gö ön nü ül l A Ay yd do o¤ ¤a an n* ** ** *, , Z Ze ey yn ne ep p K Ka ar ra ak ka afl fl* ** ** ** *, , Ü Üm mr ra ah h A Ay yd do o¤ ¤a an n* *, , A Ay yg gü ün n D Di in nd da ar r* *, , R Ru uk ki iy ye e E Ek ke er r Ö Öm me er ro o¤ ¤l lu u* * Material and Method:The study included 61 beta-thalassemia major subjects (age 4 to 20 years; mean age, 10.7±4.1 years; 32 females and 29 males) with normal left ventricular function and 52 healthy control subjects matched for age and sex. All participants underwent M-mode echocardiography and left ventricular systolic function was measured; diastolic function of the right and left ventricle was analysed using tissue Doppler imaging and conventional Doppler echocardiography. SPSS for Windows 13.0 sofware program were used for statistical analysis, and student's t-test were used to compare data. This study was approved by the ethics committee of the Istanbul Medical Faculty. Results: Body surface area was significantly smaller in the patient group than in the control group (1.0±0.2 vs. 1.2±0.3 m 2 , p<0.01). Transmitral early peak-E velocity (E) and E/A ratio was found to be significantly increased in patients in comparison to the controls (p<0.01 and p=0.039, respectively). Late peak-A velocity (A) of mitral valve and all Doppler velocities across the tricuspid valve did not differ between groups (p>0.05). In patients with beta-thalassemia major, the early diastolic (Em) velocities of the myocardium at the base of the left and right ventricle, at the middle segment of the left and right ventricle, and the interventricular septum were found to be higher than controls (p<0.05). We also found significantly higher late diastolic (Am) velocities at the base and middle segments of RV and higher Em/Am ratio at the base of the right ventricle and at the middle segments of the left ventricle, right ventricle and the interventricular septum compared with controls (p<0.05). Conclusions: These findings obtained from young aged beta-thalassemia major patients with normal ventricular systolic function were believed to arise from high preload and hyperdynamic response to chronic anemia rather than true ventricular restriction. We suggest that long-term follow-up studies should be carried out in patients with beta-thalassemia major using tissue Doppler in order to evaluate the diagnostic accuracy of this imaging technique for the diagnosis of early stages of cardiac involvement. (Turk Arch Ped 2011; 46: 27-32)

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“…A study by Parale et al evaluating diastolic cardiac functions in pediatric thalassaemia patients documented that E/Em ratio exhibited statistically signi cant elevation in the thalassaemia group [15]. A study by Silvilairat et al evaluating severity of iron deposition by tissue Doppler imaging in a pediatric patient population showed that E/Em ratio was calculated to be signi cantly elevated in the patients with mean ferritin level > 5000 ng/ml, whereas the amplitude of Sm wave was signi cantly decreased in the patients with ferritin level greater than 2500 ng/ml [16]. We identi ed in accordance with the previous studies that E/Em ratio showed statistically signi cance increase in the thalassaemia group compared with the control group in addition to Em ve Sm waves of statistically signi cant lower amplitude.…”

Section: Discussionmentioning

confidence: 99%

The relation of fragmented QRS with tissue Doppler derived parameters in patients with b-thalassaemia major

Büyükkaya

Karakaş

Kurt

et al. 2012

CIM

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Turkey e relation of fragmented QRS with tissue Doppler derived parameters in patients with b-thalassaemia major AbstractPurpose: e most important complication encountered in patients with b-thalassaemia major is degenerative brosis developing as a result of iron accumulation in myocardial tissue. Dysfunction pursues this accumulation. Recently, presence of fragmented QRS (fQRS) in ECG has been regarded as a predictor of myocardial brosis. We aimed in our study to investigate the frequency with which fQRS develops in patients with bthalassaemia major and to disclose the correlation between fQRS frequency and Dopplerderived indices.Methods: e patients with b-thalassaemia major (n=66; mean age: 23±6 years) and healthy controls (n=30; mean age: 23±4 years) were included. fQRS pattern was described as presence of RSR' manifested as existence of additional R wave and notching in either R or S waves in ECG recordings. 2D, M-mode, conventional Doppler, tissue Doppler echocardiography parameters were assessed. Mean serum ferritin levels over past 5 years were also calculated.Results: When compared to those in control group, fQRS was more frequent in bthalassaemia major group, indicating statistical signi cance (p = 0.001). While E/Em and ferritin level exhibited statistically signi cant increase in thalassaemia patients with fQRS (p < 0.05), the mean Em and Sm values were found to be signi cantly low (p < 0.05).Conclusions: fQRS was frequently observed in the patients with b-thalassaemia major, which was of statistical signi cance. Tissue Doppler-derived diastolic and systolic indices in thalassaemia cases with fQRS showed statistically signi cant impairment compared to those without fQRS. In conclusion, fQRS may represent a novel noninvasive marker for cardiac involvement in patients with b-thalassaemia major. e most important complication in patients with bthalassaemia major is cardiac dysfunction due to accumulation of iron in myocardial cells [1]. Heart failure holds the topmost position among the mortality reasons in patients with bthalassaemia major [2]. 50% of the cases with thalassaemia major are complicated with manifest heart failure, for which reason early diagnosis may prove helpful in the treatment [3,4].Recently, existence of fragmented QRS (fQRS) in ECG strips has been regarded as a predictor of myocardial brosis [5]. at fQRS proved to be a novel marker pointing out possible existence of myocardial scar was documented in the patients with coronary arterial disease [6].Various number of parameters related to conventional Doppler echocardiography was used during investigations on the cardiac functioning in the patients with b-thalassaemia until now [7]. We used in our study the tissue Doppler imaging modality which has less technical limitations compared to conventional Doppler echocardiography.What we aimed in this study was to disclose relationship between tissue Doppler echocardiography-derived indices and frequency of fQRS, a possibly novel indicator that may be used in the early identi cation of cardia...

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Tissue Doppler echocardiography reliably reflects severity of iron overload in pediatric patients with β thalassemia

Abstract: Decreased DT, increased PVAR-MVA duration, and increased E/Em ratio reliably reflected severe iron overload in pediatric patients with beta thalassemia. Systolic and diastolic LV function is preserved in patients who have serum ferritin <2500 ng/mL.

Cited by 28 publications

References 28 publications

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

Beta talasemi majorlu çocuk ve ergenlerde doku Doppler yöntemiyle diyastolik işlevlerin değerlendirilmesi

The relation of fragmented QRS with tissue Doppler derived parameters in patients with b-thalassaemia major

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