Left ventricular filling pattern in  -thalassaemia major -- a Doppler echocardiographic study

Kremastinos, Dimitrios Th.; Rentoukas, Elias; Mavrogeni, Sophie; Kyriakides, Zenon S.; Politis, C.; Toutouzas, Pavlos

doi:10.1093/eurheartj/14.3.351

Cited by 41 publications

(30 citation statements)

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“…"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). In our trans-mitral PW Doppler results, E value was found to be significantly higher and E/A ratio was found to be slightly significantly higher.…”

Section: Discussionsupporting

confidence: 41%

“…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).…”

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: Discussionsupporting

confidence: 41%

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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“…37 It should be noted that LV diastolic dysfunction and reduced LVEF may both be masked by an anemia-induced high-output state in patients with hemoglobinopathies and other hematologic conditions. 38 Thus, a pseudonormalized pattern is frequently encountered in transmitral inflow, 10 which may be unmasked by studying pulmonary vein flow pattern or mitral annulus motion by tissue Doppler imaging. On the other hand, it has been proposed that a higher cutoff value for LVEF should be applied in those patients.…”

Section: Kremastinos and Farmakis Iron Overload Cardiomyopathy 2255mentioning

confidence: 99%

Iron Overload Cardiomyopathy in Clinical Practice

2011

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“…Cardiac function in these patients remains normal for many years, but once symptoms of heart failure become evident, death usually occurs within 1 year. 1,2 Cardiac dysfunction may be aggravated by myocarditis 17 and pulmonary hypertension, 18 and iron deposition in the heart is the major determinant of survival. 3,4 Although cardiac death is the most frequent cause of death in TM patients, early markers of cardiac function with prognostic impact on survival have not yet been adequately defined.…”

Section: Discussionmentioning

confidence: 99%

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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Left ventricular filling pattern in -thalassaemia major -- a Doppler echocardiographic study

Cited by 41 publications

References 0 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

Iron Overload Cardiomyopathy in Clinical Practice

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

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