Early impairment of myocardial function in young patients with β‐thalassemia major

Magrì, Damiano; Sciomer, Susanna; Fedele, Francesco; Gualdi, Gianfranco; Casciani, Emanuele; Pugliese, Pellegrina; Anna, Lívia; Ferrazza, Giancarlo; Pasquazzi, Eleonora; Schifano, Erika; Mussino, Eleonora; Quaglione, Raffaele; Piccirillo, Gianfranco

doi:10.1111/j.1600-0609.2008.01054.x

Cited by 45 publications

(46 citation statements)

References 28 publications

(45 reference statements)

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“…Although some of these studies have demonstrated a significant correlation (25)(26)(27), others have not (11,28). The main limitation of TDI parameters is their dependence on heart rate.…”

Section: Discussionmentioning

confidence: 99%

The relation of color m-mode flow propagation with cardiac iron load in patients with thalassemia major

Özbek

Acar²,

Kayrak³

et al. 2011

Diagn Interv Radiol

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“…Although some of these studies have demonstrated a significant correlation (25)(26)(27), others have not (11,28). The main limitation of TDI parameters is their dependence on heart rate.…”

Section: Discussionmentioning

confidence: 99%

The relation of color m-mode flow propagation with cardiac iron load in patients with thalassemia major

Özbek

Acar²,

Kayrak³

et al. 2011

Diagn Interv Radiol

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“…12 Tissue Doppler imaging (TDI) analysis disclosed, in these patients, an early myocardial dysfunction likely to be related specifically to this initial iron overload, as suggested by the correlation with cardiac T2* values. 13 In our study we could not evaluate patients by cardiac MRI or TDI, which becomes the main limitation of this study.…”

Section: Discussionmentioning

confidence: 91%

Marcadores eletrocardiográficos para detecção precoce de doença cardíaca em pacientes com talassemia beta maior

Nişli¹,

Yavuz²,

Naci³

et al. 2010

J. Pediatr. (Rio J.)

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Objective: To comparatively evaluate P-wave dispersion (PWD) in patients with β-thalassemia major (TM) and healthy control subjects for the early prediction of arrhythmia risk.Methods: Eighty-one children with β-TM, aged 4-19 years, and 74 healthy children (control group) underwent routine electrocardiography and transthoracic echocardiography for cardiac evaluation. PWD was calculated as the difference between the maximum and the minimum P-wave duration.Results: There was a statistically significant difference between study and control groups in peak early (E) mitral inflow velocity and E/late (A) velocity ratio. Maximum P-wave duration and PWD were found to be significantly higher in β-TM patients than in control subjects. Conclusions:Increased PWD in our β-TM patients might be related to depression of intra-atrial conduction due to atrial dilatation and increased sympathetic activity. These patients should be closely followed up for risk of lifethreatening arrhythmias.J Pediatr (Rio J). 2010;86(2):159-162: Thalassemia major, P-wave dispersion, ferritin, cardiac involvement. Original article0021-7557/10/86-02/159 Jornal de PediatriaCopyright © 2010 by Sociedade Brasileira de Pediatria IntroductionCardiac failure and sudden death, the latter probably due to arrhythmias, remain the major causes of death in β-thalassemia major (TM). 1 Disease prognosis, however, has been modified with regular blood transfusions and iron chelation therapy with deferoxamine. 2 P-wave dispersion (PWD) is a simple electrocardiographic marker that has been reported to be associated with inhomogeneous and discontinuous propagation of sinus impulses. It has been defined as the difference between the maximum and the minimum P-wave duration. 3-4 Prolonged P-wave duration and increased PWD have been shown to carry an increased risk for atrial fibrillation. 4 Cardiomyopathy is associated with a four-to six-fold increase in the risk of developing atrial fibrillation. 5 The present study aimed to comparatively investigate these electrocardiographic markers in patients with β-TM and healthy control subjects for the early prediction of arrhythmia risk. MethodsThe study population consisted of two groups: Group I -81 children with β-TM; and group II -74 healthy children Peak mitral inflow velocities in early diastole (E) and after atrial contraction (A) were expressed as cm/s. Deceleration time of peak E filling velocity was measured in milliseconds.Overall LV diastolic dysfunction was defined as the presence of any abnormality of the mitral valve. Twelve-lead electrocardiogram (ECG) was recordedfor each patient and control subject at a rate of 50 mm/s.At the time of electrocardiographic recording, all subjects were in sinus rhythm and none were taking any type of antiarrhythmic agent. P-wave duration was measured manually by two of the investigators who were blinded to the participants' clinical status. All measurements were performed using calipers and magnifying lens to improve accuracy. The onset of P-wave was defined as the junctio...

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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: 81%

“…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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Early impairment of myocardial function in young patients with β‐thalassemia major

Cited by 45 publications

References 28 publications

The relation of color m-mode flow propagation with cardiac iron load in patients with thalassemia major

The relation of color m-mode flow propagation with cardiac iron load in patients with thalassemia major

Marcadores eletrocardiográficos para detecção precoce de doença cardíaca em pacientes com talassemia beta maior

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

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