Prognostic Significance of Left Ventricular Diastolic Indexes in β-Thalassemia Major

Hou, Jia‐Woei; Wu, Mei‐Hwan; Lin, Kai‐Hsin; Lue, Hung-Chi

doi:10.1001/archpedi.1994.02170080092018

Cited by 19 publications

(20 citation statements)

References 14 publications

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“…Overall, one-third of all patients with evidence of abnormal diastolic function on echocardiography had anaemia. This may be partly attributable to an increase in LV mass observed in patients with anaemia [31], similar to findings in patients with β-thalassemia, where an increase in LV mass is associated with increased cardiac stiffness [14]. However, the overall rate of LVH was not significantly different between diabetic patients without and with anaemia.…”

Section: Discussionsupporting

confidence: 72%

“…Over time functional changes occur, with impaired LV relaxation and compliance [13]. For example, diastolic dysfunction in patients with asymptomatic β-thalassaemia is correlated with the severity of anaemia and ultimately with adverse cardiac outcomes [14]. We hypothesize that anaemia is also associated with early cardiac dysfunction in patients with Type II diabetes.…”

Section: Introductionmentioning

confidence: 98%

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Diastolic dysfunction is associated with anaemia in patients with Type II diabetes

et al. 2005

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Anaemia is common in patients with diabetes and associated with an increased risk of diabetic complications. Although the role of anaemia in heart failure is established, we hypothesize that anaemia also contributes to an increased risk of cardiac dysfunction in patients with Type II diabetes. In the present study, 228 consecutive adults with diabetes were investigated using transthoracic echocardiography. Echocardiographic parameters were correlated with the Hb (haemoglobin) level and adjusted for other risk factors for cardiac dysfunction using multivariate analysis. More than one in five patients (23%) had anaemia, which was an independent risk factor for cardiac dysfunction on echocardiography. Over one-third of all patients with evidence of abnormal cardiac function (diastolic and/or systolic dysfunction) on echocardiography had anaemia compared with <5% of patients with normal echocardiographic findings. Most patients with anaemia had cardiac dysfunction (94%), with the major abnormality being diastolic dysfunction associated with an increased left ventricular mass and impaired relaxation indices. A continuous association between diastolic function and Hb was also observed in patients without anaemia. In patients with a history of cardiovascular disease, systolic dysfunction was twice as common in patients with anaemia. Anaemia was also correlated with plasma markers of cardiac risk, including BNP (brain natriuretic peptide), CRP (C-reactive protein) and AVP (arginine vasopressin). Notably, the predictive utility of these markers was eliminated after adjusting for Hb. Consequently, the inexpensive measurement of Hb may be a useful tool to identify diabetic patients at increased risk of cardiac dysfunction.

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Section: Discussionsupporting

confidence: 72%

Section: Introductionmentioning

confidence: 98%

Diastolic dysfunction is associated with anaemia in patients with Type II diabetes

et al. 2005

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“…Monitoring cardiac function was shown to have prognostic value on symptomatic cardiac disease and death in three retrospective analytical studies, 35,47,48 while in another study it was not. 49 The validity of the studies was judged to be good for the number of patients and for the follow-up, but poor for the definition of cardiac outcomes.…”

Section: Monitoring Iron Chelationmentioning

confidence: 99%

“…Since the panel identified SF, LIC and heart T2* MRI as monitoring procedures, the literature revision was addressed to studies that compared the prognosis of patients at different values of these tests. [33][34][35][36][45][46][47][48][49] Only non-prospective studies, with an uncontrolled or unblinded design, and in which the cut-off values of the test were determined without test performance analysis, were available. The panel reconciled the discrepant results according to principles of good practice.…”

Section: Switching To An Alternative Iron Chelation Therapy In Patienmentioning

confidence: 99%

Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders

Angelucci¹,

Barosi²,

Camaschella³

et al. 2008

Haematologica

179

156

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New measures of iron accumulation in liver and heart (superconducting quantum inference device and magnetic resonance imaging), and oral iron chelators (deferiprone and deferasirox) are available for managing iron overload in thalassemia major. To assure appropriate use of these new health technologies, the Italian Society of Hematology appointed a panel of experts to produce clinical practiceguidelines for the management of iron overload in thalassemia major and related disorders. The analytical hierarchy process, a technique for multicriteria decision analysis, was applied to relevant key questions in order to identify the alternative strategies, generate explicit criteria for their evaluation, and check how well the alternatives fulfilled the criteria. The result of a comprehensive systematic review of articles released from 1990 to 2007 was used as a source of scientific evidence to compare the decisional options pairwise, and select the final recommendation. Every step in the model was developed from questionnaires and group discussion. The resulting recommendations advise about which examination to carry out in order to plan iron chelation therapy, when to start iron chelation, which iron chelator to choose in regularly transfused patients, how to monitor iron chelation therapy, and when and how to switch standard therapy.

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“…Initiating therapy with converting-enzyme inhibitors in patients with LVRFP is under consideration (preliminary results of our group are encouraging). 20 In another 5-year follow-up Doppler study, the presence of an abnormal total diastolic period and the duration of the early diastolic flowvelocity peak values correlated well with prognosis in transfusion-dependent TM patients with normal systolic function. 21 In the present study 6 of 11 deceased patients had symptomatic systolic heart failure preceding cardiac death.…”

Section: Discussionmentioning

confidence: 86%

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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Prognostic Significance of Left Ventricular Diastolic Indexes in β-Thalassemia Major

Cited by 19 publications

References 14 publications

Diastolic dysfunction is associated with anaemia in patients with Type II diabetes

Diastolic dysfunction is associated with anaemia in patients with Type II diabetes

Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders

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

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