Myocardial scarring by delayed enhancement cardiovascular magnetic resonance in thalassaemia major

Pepe, Alessia; Positano, Vincenzo; Capra, Marcello; Maggio, Aurelio; Pinto, Carmela Lo; Spasiano, Anna; Forni, Gianluca; Derchi, Giorgio; Favilli, Brunella; Rossi, Giuseppe; Cracolici, Eliana; Midiri, Massimo; Lombardi, Massimo

doi:10.1136/hrt.2008.156497

Cited by 79 publications

(57 citation statements)

References 30 publications

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“…Those findings were subsequently confirmed in larger patient populations. 34,35 Moreover, CMR also revealed evidence of myocardial fibrosis and scars in thalassemia major patients with iron overload, 36 although the finding was later questioned by other investigators. 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.…”

Section: Kremastinos and Farmakis Iron Overload Cardiomyopathy 2255mentioning

confidence: 98%

Iron Overload Cardiomyopathy in Clinical Practice

2011

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Section: Kremastinos and Farmakis Iron Overload Cardiomyopathy 2255mentioning

confidence: 98%

Iron Overload Cardiomyopathy in Clinical Practice

2011

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“…Risk factors for heart disease (smoking, family history, hypertension, diabetes mellitus type 1 and 2, dyslipidemia, obesity) were also considered, since previous studies showed the presence of coronary artery disease in patients with thalassemia major 2 and, moreover, cardiac risk factors were reported to be significantly correlated with myocardial fibrosis. 14 We performed a retrospective review of the MRI results and of clinical data in thalassemia major patients who had undergone at least one cardiac MRI examination at the time of the study. For patients who had undergone more than one MRI, the results of the first examination were considered in order to avoid the effect of intensive chelation started in some patients found to have a low T2*.…”

Section: Design and Methodsmentioning

confidence: 99%

Cardiac iron and cardiac disease in males and females with transfusion-dependent thalassemia major: a T2* magnetic resonance imaging study

Marsella¹,

Borgna‐Pignatti²,

Meloni³

et al. 2011

Haematologica

113

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“…Historical postmortem studies showed severe replacement cardiac fibrosis, 27,72 but this is now rare in more modern cohorts of patients dying of HF. 73 More minor patches of myocardial fibrosis have been identified in vivo with late gadolinium-enhancement CMR in Italian patients with TM, 77 but this has not been reproduced in the United Kingdom. 78 This difference probably results from higher levels of myocarditis resulting from hepatitis C infection in Italy.…”

Section: Clinical Cardiac Manifestations Of Iron Overloadmentioning

confidence: 99%

Cardiovascular Function and Treatment in β-Thalassemia Major

Pennell¹,

Udelson²,

Arai³

et al. 2013

Circulation

324

172

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This aim of this statement is to report an expert consensus on the diagnosis and treatment of cardiac dysfunction in β-thalassemia major (TM). This consensus statement does not cover other hemoglobinopathies, including thalassemia intermedia and sickle cell anemia, in which a different spectrum of cardiovascular complications is typical. There are considerable uncertainties in this field, with a few randomized controlled trials relating to treatment of chronic myocardial siderosis but none relating to treatment of acute heart failure. The principles of diagnosis and treatment of cardiac iron loading in TM are directly relevant to other iron-overload conditions, including in particular Diamond-Blackfan anemia, sideroblastic anemia, and hereditary hemochromatosis. Heart failure is the most common cause of death in TM and primarily results from cardiac iron accumulation. The diagnosis of ventricular dysfunction in TM patients differs from that in nonanemic patients because of the cardiovascular adaptation to chronic anemia in non–cardiac-loaded TM patients, which includes resting tachycardia, low blood pressure, enlarged end-diastolic volume, high ejection fraction, and high cardiac output. Chronic anemia also leads to background symptomatology such as dyspnea, which can mask the clinical diagnosis of cardiac dysfunction. Central to early identification of cardiac iron overload in TM is the estimation of cardiac iron by cardiac T2* magnetic resonance. Cardiac T2* <10 ms is the most important predictor of development of heart failure. Serum ferritin and liver iron concentration are not adequate surrogates for cardiac iron measurement. Assessment of cardiac function by noninvasive techniques can also be valuable clinically, but serial measurements to establish trends are usually required because interpretation of single absolute values is complicated by the abnormal cardiovascular hemodynamics in TM and measurement imprecision. Acute decompensated heart failure is a medical emergency and requires urgent consultation with a center with expertise in its management. The first principle of management of acute heart failure is control of cardiac toxicity related to free iron by urgent commencement of a continuous, uninterrupted infusion of high-dose intravenous deferoxamine, augmented by oral deferiprone. Considerable care is required to not exacerbate cardiovascular problems from overuse of diuretics or inotropes because of the unusual loading conditions in TM. The current knowledge on the efficacy of removal of cardiac iron by the 3 commercially available iron chelators is summarized for cardiac iron overload without overt cardiac dysfunction. Evidence from well-conducted randomized controlled trials shows superior efficacy of deferiprone versus deferoxamine, the superiority of combined deferiprone with deferoxamine versus deferoxamine alone, and the equivalence of deferasirox versus deferoxamine.

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Myocardial scarring by delayed enhancement cardiovascular magnetic resonance in thalassaemia major

Cited by 79 publications

References 30 publications

Iron Overload Cardiomyopathy in Clinical Practice

Iron Overload Cardiomyopathy in Clinical Practice

Cardiac iron and cardiac disease in males and females with transfusion-dependent thalassemia major: a T2* magnetic resonance imaging study

Cardiovascular Function and Treatment in β-Thalassemia Major

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