β-Thalassemia Major (βTM) is a hereditary chronic haemolytic anaemia that requires multiple blood transfusions all along lifetime. A complication of this treatment is iron overload (IO), mostly located on the reticulo-endothelial system, joints, endocrine glands, liver and the heart. Iron overload has also been found to be responsible for up to 71% of cardiac deaths in βTM patients, as a possible consequence of congestive heart failure. However, in the last few years, intensive treatment with iron chelating agents has strongly decreased the outcomes' rate, at least in patients with no evidence of overt cardiac failure. Echocardiography is known to be a powerful technique for the assessment of preclinical markers of left ventricular (LV) dysfunction in patients with suspected or certain myocardial IO. More recently, tissue Doppler velocity imaging (TDVI), a new ultrasound technique, has been demonstrated to improve the recognition of LV function much better than conventional Doppler study, but not yet univocal data are available in the literature for βTM patients. Over the last decade, cardiac magnetic resonance imaging (MRI) has become the gold standard for myocardial tissue analysis in several cardiac diseases. A specific technological implementation called T2 star is now available in order to provide a strong marker of myocardial IO, as expression of myocardial relaxation time. In this pilot study, we sought to investigate whether TDVI and/or conventional Doppler echocardiography can discriminate βTM patients with impaired T2star value, in the absence of clinical signs of cardiac failure.

On the use ofconventional and tissue Doppler echocardiography in patients withbeta-Thalassemia major and myocardial iron-overload: Preliminary data by a singlecentre study.

SALPIETRO DAMIANO, Carmelo;
2009-01-01

Abstract

β-Thalassemia Major (βTM) is a hereditary chronic haemolytic anaemia that requires multiple blood transfusions all along lifetime. A complication of this treatment is iron overload (IO), mostly located on the reticulo-endothelial system, joints, endocrine glands, liver and the heart. Iron overload has also been found to be responsible for up to 71% of cardiac deaths in βTM patients, as a possible consequence of congestive heart failure. However, in the last few years, intensive treatment with iron chelating agents has strongly decreased the outcomes' rate, at least in patients with no evidence of overt cardiac failure. Echocardiography is known to be a powerful technique for the assessment of preclinical markers of left ventricular (LV) dysfunction in patients with suspected or certain myocardial IO. More recently, tissue Doppler velocity imaging (TDVI), a new ultrasound technique, has been demonstrated to improve the recognition of LV function much better than conventional Doppler study, but not yet univocal data are available in the literature for βTM patients. Over the last decade, cardiac magnetic resonance imaging (MRI) has become the gold standard for myocardial tissue analysis in several cardiac diseases. A specific technological implementation called T2 star is now available in order to provide a strong marker of myocardial IO, as expression of myocardial relaxation time. In this pilot study, we sought to investigate whether TDVI and/or conventional Doppler echocardiography can discriminate βTM patients with impaired T2star value, in the absence of clinical signs of cardiac failure.
2009
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11570/1886347
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