Detection of Early Cardiac Dysfunction in Patients with Beta Thalassemia by Tissue Doppler Echocardiography

Marcì, Marcello; Pitrolo, Lorella; Pinto, Carmela Lo; Sanfilippo, Nicola; Malizia, R

doi:10.1111/j.1540-8175.2010.01270.x

Cited by 13 publications

(12 citation statements)

References 29 publications

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“…Regular clinical follow-up is strongly recommended for precocious detection of symptoms and signs of myocardial dysfunction [15]. On the hypothesis that longitudinal ventricular function may give early information about impending ventricular function, we used TVI and S/SRI measurements [6,7].…”

Section: Discussionmentioning

confidence: 99%

Detection of Left Ventricular Regional Function in Asymptomatic Children with beta-Thalassemia Major by Longitudinal Strain and Strain Rate Imaging

Bay¹,

Başpınar²,

Leblebisatan³

et al. 2013

Tjh

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Objective: Cardiac failure due to iron overload remains the most common cause of death in patients with beta-thalassemia major. This study aimed to evaluate myocardial function in children with beta-thalassemia major using standard echocardiography technique and strain rate imaging.Materials and Methods: Conventional echocardiographic analysis, tissue velocity imaging, and strain/strain rate imaging of the left ventricle were evaluated in 48 children with beta-thalassemia major (19 girls, 29 boys; 8.39±4.05 years) and 22 healthy children (11 girls, 11 boys; 8±3.72 years). Results: Conventional echocardiographic examinations revealed that beta-thalassemia patients had larger left ventricular end-systolic diameter, end-diastolic and end-systolic volume, left ventricular mass index, and mitral early/late diastolic flow velocity ratio (p<0.05). Strain and strain rate imaging study of the basal lateral wall of the left ventricle was higher in patients than in controls, at p=0.035 and p=0.008, respectively. Conclusion: We found that superior systolic strain and strain rate imaging of the left ventricle indicated the presence of regional systolic function in the left ventricular wall. We suggest that left ventricle volume and mass index parameters might be more sensitive than the other conventional and strain/strain rate imaging parameters during childhood. However, the adulthood strain and strain rate imaging values may be lower than controls, exceeding the critical level of iron overload.Conflict of interest:None declared.

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Section: Discussionmentioning

confidence: 99%

Detection of Left Ventricular Regional Function in Asymptomatic Children with beta-Thalassemia Major by Longitudinal Strain and Strain Rate Imaging

Bay¹,

Başpınar²,

Leblebisatan³

et al. 2013

Tjh

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“…Kremastinos et al reported in their study on young and adult thalassaemia patients that evaluation of diastolic functions of the heart by conventional Doppler echocardiography revealed similar parameters like E/A, IVRT and DT to those in the control group [12]. A study by Marci et al documented similar E/A and DT values to those in the control group in the evaluation of diastolic functions of the heart via conventional Doppler echocardiography [13]. In accordance with the aforementioned data, we also did not identify any distinction with regard to conventional Doppler echocardiography ndings between the groups.…”

Section: Discussionmentioning

confidence: 52%

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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“…81 (4) Impaired diastolic function as shown by tissue Doppler imaging has been reported with cardiac iron overload, but only in small studies, and its low sensitivity limits its use for diagnosis and as a prognostic tool. 85,86 Impaired diastolic function shown by CMR also had low sensitivity for identification of cardiac iron loading. 87 …”

Section: Clinical Cardiac Manifestations Of Iron Overloadmentioning

confidence: 99%

Cardiovascular Function and Treatment in β-Thalassemia Major

Pennell¹,

Udelson²,

Arai³

et al. 2013

Circulation

315

172

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This aim of this statement is to report an expert consensus on the diagnosis and treatment of cardiac dysfunction in β-thalassemia major (TM). This consensus statement does not cover other hemoglobinopathies, including thalassemia intermedia and sickle cell anemia, in which a different spectrum of cardiovascular complications is typical. There are considerable uncertainties in this field, with a few randomized controlled trials relating to treatment of chronic myocardial siderosis but none relating to treatment of acute heart failure. The principles of diagnosis and treatment of cardiac iron loading in TM are directly relevant to other iron-overload conditions, including in particular Diamond-Blackfan anemia, sideroblastic anemia, and hereditary hemochromatosis. Heart failure is the most common cause of death in TM and primarily results from cardiac iron accumulation. The diagnosis of ventricular dysfunction in TM patients differs from that in nonanemic patients because of the cardiovascular adaptation to chronic anemia in non–cardiac-loaded TM patients, which includes resting tachycardia, low blood pressure, enlarged end-diastolic volume, high ejection fraction, and high cardiac output. Chronic anemia also leads to background symptomatology such as dyspnea, which can mask the clinical diagnosis of cardiac dysfunction. Central to early identification of cardiac iron overload in TM is the estimation of cardiac iron by cardiac T2* magnetic resonance. Cardiac T2* <10 ms is the most important predictor of development of heart failure. Serum ferritin and liver iron concentration are not adequate surrogates for cardiac iron measurement. Assessment of cardiac function by noninvasive techniques can also be valuable clinically, but serial measurements to establish trends are usually required because interpretation of single absolute values is complicated by the abnormal cardiovascular hemodynamics in TM and measurement imprecision. Acute decompensated heart failure is a medical emergency and requires urgent consultation with a center with expertise in its management. The first principle of management of acute heart failure is control of cardiac toxicity related to free iron by urgent commencement of a continuous, uninterrupted infusion of high-dose intravenous deferoxamine, augmented by oral deferiprone. Considerable care is required to not exacerbate cardiovascular problems from overuse of diuretics or inotropes because of the unusual loading conditions in TM. The current knowledge on the efficacy of removal of cardiac iron by the 3 commercially available iron chelators is summarized for cardiac iron overload without overt cardiac dysfunction. Evidence from well-conducted randomized controlled trials shows superior efficacy of deferiprone versus deferoxamine, the superiority of combined deferiprone with deferoxamine versus deferoxamine alone, and the equivalence of deferasirox versus deferoxamine.

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Detection of Early Cardiac Dysfunction in Patients with Beta Thalassemia by Tissue Doppler Echocardiography

Abstract: Our study suggests that mitral annular systolic velocity <7.9 cm/s is associated to the onset of adverse cardiac events.

Cited by 13 publications

References 29 publications

Detection of Left Ventricular Regional Function in Asymptomatic Children with beta-Thalassemia Major by Longitudinal Strain and Strain Rate Imaging

Detection of Left Ventricular Regional Function in Asymptomatic Children with beta-Thalassemia Major by Longitudinal Strain and Strain Rate Imaging

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

Cardiovascular Function and Treatment in β-Thalassemia Major

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