An angiotensin-converting enzyme inhibitor improves left ventricular systolic and diastolic function in transfusion-dependent patients with β-thalassemia major

Karvounis, Haralambos; Zaglavara, Theodora; Parharidis, Georgios E.; Nouskas, Ioannis; Hassapopoulou, Eleni P.; Gemitzis, Konstantinos D.; Louridas, Georgios E.

doi:10.1067/mhj.2001.112090

Cited by 18 publications

(16 citation statements)

References 24 publications

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“…In literature, comparisons of left ventricular M-mode measurements in patients with thalassemia and in healthy controls have been made without measuring body surface areas of the patients or without considering body surface areas. Therefore, left ventricular M-mode measurements may give different results in these studies conducted in subjects who have not yet developed heart failure (22,(24)(25)(26)(27)(28) …”

Section: Discussionmentioning

confidence: 99%

“…In literature, contradictory results have been reported in studies in which trans-mitral measurements have been made with PW Doppler in patients with thalassemia major without signs of heart failure (22,(24)(25)(26)(27)(28)(29). "Restrictive" diastolic dysfunction was found in some studies conducted in thalassemia major patients having normal left ventricular systolic function without signs of heart failure (24,25). In other studies, "restrictive" dysfunction was not reported, although elevation in trans-mitral E wave or differences in other diastolic indicators were found (22,(26)(27)(28)(29).…”

Section: Discussionmentioning

confidence: 99%

“…Studies conducted with PW Doppler in thalassemia patients until this time have predominantly examined left ventricular function (22,24,25,(27)(28)(29)(30). Very few studies have examined right ventricular diastolic function (32)(33)(34).…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Yavuz¹,

Nişli²,

Ertuğrul³

et al. 2011

tpa

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“…5 They found LVRFP in 50% of asymptomatic transfusion-dependent TM patients with normal LV systolic function, but others have not found LVRFP in TM patients, except for a small minority of advanced age patients with elevated serum ferritin levels. 6,19 Our study group comprised 45 asymptomatic adult patients with normal LV dimensions and systolic function, who were not on any cardioactive medication. A large proportion of them (42.2%) had LVRFP at the time of the first echocardiographic examination.…”

Section: Discussionmentioning

confidence: 99%

“…None had an abnormal relaxation filling pattern of the LV. There was no statistically significant differences at the start of the study between patients with LVRFP and patients with LV non-RFP regarding the mean values of age (19 The baseline Doppler-echocardiographic data of the patients with LVRFP and patients with LV non-RFP is shown in Table 1. During the follow-up period, only 8 of 26 patients (31%) with LV non-RFP changed to LVRFP.…”

Section: Hematological Profile and Echocardiographic Datamentioning

confidence: 96%

Survival in Thalassaemia Major Patients Prognostic Value of Doppler-Demonstrated Left Ventricular Restrictive Filling Pattern

Efthimiadis

Hassapopoulou

Tsikaderis

et al. 2006

Circ J

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ongestive heart failure is the main cause of death in transfusion-dependent homozygous -thalassaemia (thalassaemia major (TM)) patients. When symptoms of heart failure appear, death usually occurs within 1 year. 1,2 Although cardiac death is the most common cause of death in patients with TM, early markers of cardiac function with prognostic impact on survival have not yet been adequately defined. Deposition of iron in the heart is the major determinant of survival in these patients. 3,4 Left ventricular (LV) restrictive filling pattern (RFP), as assessed by Doppler-echocardiography, is a well-recognized feature of TM patients with normal LV systolic function, 5 although debated by some authors. 6,7 LVRFP, as assessed by Doppler-echocardiography, is characterized by an increase of the peak early to peak late filling velocity ratio (>2) and/or shortened deceleration time of early filling (<150 ms) because of impaired ventricular compliance. 8,9 Doppler-demonstrated LVRFP is an ominous sign in a variety of cardiac diseases with concomitant systolic heart failure, 10-13 but the clinical significance of restrictive physiology in TM patients has not yet been well established.The purpose of this longitudinal study was to investigate Circulation Journal Vol.70, August 2006the impact of the Doppler-demonstrated LV filling pattern and also the impact of compliance with chelation therapy on survival in a cohort of asymptomatic TM adult patients with normal LV systolic function throughout a 15-year observation period. Methods Patients and Study ProtocolAll patients gave written informed consent and the protocol of this study was reviewed and approved by the institutional review board. Sixty-five consecutive TM patients, who were followed-up in the Thalassaemia Unit, were considered candidates for the analysis. All patients initially underwent clinical evaluation, chest X-ray, 12-lead ECG and Doppler-echocardiography from May 1989 to August 1989, in order to obtain baseline data, and then they were examined periodically by clinical examination and Dopplerechocardiography up to the end of the data collection (May 2004). The endpoint of the study was death from cardiovascular causes. Patients' inclusion criteria were: (1) age ≥14 years at the initial examination, (2) normal LV end-diastolic diameter (=55 mm) and normal LV systolic function (fractional shortening =30%), as assessed by echocardiography and (3) absence of any symptom of cardiac and/or pulmonary pathology.Among the 65 patients initially evaluated, 20 were excluded for the following reasons: 11 were <14 years of age; 4 had LV fractional shortening <30%; 5 died from non-car- Methods and ResultsThe study group comprised 45 asymptomatic transfusion-dependent patients with TM and normal LV systolic function. All patients were chelated with desferrioxamine. They were regularly evaluated by clinical and Doppler-echocardiographic studies throughout the 15-year follow-up period. The patients were categorized into 2 groups according to baseline data: those with LVRFP an...

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The efficacy of tissue Doppler imaging in predicting myocardial iron load in patients with beta-thalassemia major: correlation with T2* cardiovascular magnetic resonance

Aypar

Alehan

Hazırolan

et al. 2010

Int J Cardiovasc Imaging

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Tissue Doppler imaging (TDI) can detect myocardial dysfunction related to iron load in patients with beta-thalassemia major (TM). We aimed to assess the efficacy of pulsed-wave TDI (PW-TDI) in predicting myocardial iron load in patients with TM using T2* magnetic resonance (MR) as the gold-standard non-invasive diagnostic test. 33 asymptomatic TM patients, mean aged 18 +/- 6 years (6-31) with normal left ventricular (LV) global systolic function were evaluated by conventional echocardiography and PW-TDI. Results were compared with 20 age and sex-matched controls. TDI measures included myocardial systolic (Sm), early (Em) and late (Am) diastolic velocities at basal and middle segments of septal and lateral LV wall. Myocardial iron deposition were measured in 29/33 patients by T2* MR. TM patients were also subgrouped according to those with iron load (T2* 20 ms). Mean T2* was 12.3 +/- 7.8 ms (4-31.3). Abnormal myocardial iron load (T2* < 20 ms) was found in 25/29 (86%) patients. The following TDI measures were lower in patients than in controls: basal septal Em (P < 0.001) and Am (P < 0.05), mid-septal Am (P < 0.05), mid-lateral LV wall Sm (P < 0.05) and Am (P < 0.05). Regional myocardial dysfunction were more prominent in patients with T2*

show abstract

An angiotensin-converting enzyme inhibitor improves left ventricular systolic and diastolic function in transfusion-dependent patients with β-thalassemia major

Cited by 18 publications

References 24 publications

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

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

Survival in Thalassaemia Major Patients Prognostic Value of Doppler-Demonstrated Left Ventricular Restrictive Filling Pattern

The efficacy of tissue Doppler imaging in predicting myocardial iron load in patients with beta-thalassemia major: correlation with T2* cardiovascular magnetic resonance

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