Myocardial fibrosis by late gadolinium enhancement cardiac magnetic resonance and hepatitis C virus infection in thalassemia major patients

Pepe, Alessia; Meloni, Antonella; Borsellino, Zelia; Cuccia, Liana; Borgna‐Pignatti, Caterina; Maggio, Aurelio; Restaino, Gennaro; Gagliardotto, Francesco; Caruso, Vincenzo; Spasiano, Anna; Filosa, Aldo; Centra, Michele; D’Ascola, Domenico Giuseppe; Quarta, Antonella; Peluso, Angelo; Midiri, Massimo; Rossi, Giuseppe; Positano, Vincenzo; Capra, Marcello

doi:10.2459/jcm.0000000000000278

Cited by 25 publications

(27 citation statements)

References 32 publications

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“…The EF of both ventricles is increased in adult TM patients: 71.0 ± 6.1% in males without significant iron overload and 75.1 ± 5.9% in females . These values were considerably increased compared to matched controls (64.9 ± 5.0% in males; P < 0.001 vs. patients and 67.1 ± 4.3% in female control; P < 0.001 vs. patients) . By 7–10 years of age, significant cardiac iron overload from repeated blood transfusions may be revealed by T2* in patients with nonoptimal chelation therapy .…”

Section: Heart Failurementioning

confidence: 91%

“…In older patients, the chronic residual anemia despite transfusions induces decreased afterload (vasodilatation) and increased preload, leading to asymptomatic dilatation of the cardiac chambers and an increase in EF . The EF of both ventricles is increased in adult TM patients: 71.0 ± 6.1% in males without significant iron overload and 75.1 ± 5.9% in females . These values were considerably increased compared to matched controls (64.9 ± 5.0% in males; P < 0.001 vs. patients and 67.1 ± 4.3% in female control; P < 0.001 vs. patients) .…”

Section: Heart Failurementioning

confidence: 94%

“…Although substantial fibrosis formation was well described in iron‐overloaded TM patients in early literature 50 years ago, such significant myocardial fibrosis is now rarely seen. Fibrosis in TM has been reported as small and infrequent, but is more common in other studies, which may relate to concomitant hepatitic C myocarditis . In general, therefore, late gadolinium enhancement imaging is not routinely performed in these patients unless myocarditis or infarction is suspected, and this procedure adds significantly to the cost and duration of CMR, which is problematic in developing countries.…”

Section: Assessing the Heart In β‐Thalassemia Major: The Role Of Cmrmentioning

confidence: 99%

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Cardiac complications in thalassemia major

Auger

Pennell

2016

Annals of the New York Academy of Sciences

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The myocardium is particularly susceptible to complications from iron loading in thalassemia major. In the first years of life, severe anemia leads to high-output cardiac failure and death if not treated. The necessary supportive blood transfusions create loading of iron that cannot be naturally excreted, and this iron accumulates within tissues, including the heart. Free unbound iron catalyzes the formation of toxic hydroxyl radicals, which damage cells and cause cardiac dysfunction. Significant cardiac siderosis may present by the age of 10 and may lead to acute clinical heart failure, which must be treated urgently. Atrial fibrillation is the most frequently encountered iron-related arrhythmia. Iron chelation is effective at removing iron from the myocardium, at the expense of side effects that hamper compliance to therapy. Monitoring of myocardial iron content is mandatory for clinical management of cardiac risk. T2* cardiac magnetic resonance measures myocardial iron and is the strongest biomarker for prediction of heart failure and arrhythmic events. It has been calibrated to human myocardial tissue iron concentration and is highly reproducible across all magnetic resonance scanner vendors. As survival and patient age increases, endothelial dysfunction and diabetes may become new factors in the cardiovascular health of thalassemia patients. Promising new imaging technology and therapies could ameliorate the long-term prognosis.

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Section: Heart Failurementioning

confidence: 91%

Section: Heart Failurementioning

confidence: 94%

Section: Assessing the Heart In β‐Thalassemia Major: The Role Of Cmrmentioning

confidence: 99%

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Cardiac complications in thalassemia major

Auger

Pennell

2016

Annals of the New York Academy of Sciences

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“…11 Although a relatively uncommon phenomenon, myocardial fibrosis has been reported in as high as 15%-21% of TDT patients in some studies. 12 …”

Section: Risk Factors Of Cardiac Disease In Transfusion Dependent Thamentioning

confidence: 99%

“…12 Presence of other transfusion related comorbidities such as diabetes, hepatic disease, and other endocrinopathies such as thyroid disease may pave the way to the heart disease in TDT. 14 In particular, TDT patients affected with diabetes mellitus (DM) showed significantly higher possibilities for developing heart failure, arrhythmia, and cardiac fibrosis.…”

Section: Cardiac Hemosiderosis In Transfusion Dependentmentioning

confidence: 99%

Cardiac Hemosiderosis in Transfusion DependentThalassemia: A Mini-Review

Bazi¹,

Keramati²,

Shahramian³

2017

Int J Basic Sci Med

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Iron toxicity within cardiomyocytes is considered as the main pathogenesis of cardiac dysfunction in transfusion dependent thalassemia (TDT). Various methods such as measuring serum ferritin, evaluating cardiac functional and structural parameters by either cardiac magnetic resonance imaging (CMRI) or echocardiography, and monitoring the heart rate variability (HRV) have been proposed to monitor cardiac iron content in patients. High inconsistency is present regarding predictability of various parameters derived by each of these methods in order to predict the cardiac iron overload. The aim of present review was to grasp the most appropriate parameters predicting cardiac hemosiderosis in TDT. Predicating values for cardiac iron deposition of the most in-use indicators such as ferritin, cardiac T2* relaxation time, left ventricular ejection fraction (LVEF), and HRV were discussed. In addition, a description on the most effective preventive measures for cardiac hemosiderosis was provided.

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Biventricular Reference Values by Body Surface Area, Age, and Gender in a Large Cohort of Well‐Treated Thalassemia Major Patients Without Heart Damage Using a Multiparametric CMR Approach

Meloni

Righi²,

Missere

et al. 2020

Magnetic Resonance Imaging

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Background: Cardiac MRI plays a critical role in the management of thalassemic patients. No accurate biventricular reference values are available. Purpose: To establish the ranges for normal left ventricular (LV) and right ventricular (RV) volumes and ejection fraction (EF) and LV mass normalized to body surface area (BSA), age, and gender in a large cohort of well-treated betathalassemia major (β-TM) patients without heart damage using a multiparametric MRI. Study Type: Retrospective/cohort study. Population: In all, 251 β-TM patients with no known risk factors or cardiac disease, normal electrocardiogram, no macroscopic myocardial fibrosis, and all cardiac segments with T 2 * ≥20 msec, and 246 healthy subjects. Field Strength/Sequence: 1.5T/cine steady-state free precession (SSFP), gradient-echo T 2 *, late gadolinium enhancement (LGE) images. Assessment: Biventricular end-diastolic volume, end-systolic volume, stroke volume, and LV mass were normalized to BSA (EDVI, ESVI, SVI). Statistical Tests: Comparisons between the two groups was performed with two-samples t-test or Wilcoxon's signed rank test. For more than two groups, one-way analysis of variance (ANOVA) or a Kruskal-Wallis test were applied. Results: Compared to controls, males with β-TM showed significantlt higher LVEDVI in all the age groups, while for the other volumes the difference was significant only within one or more age groups. In females the volumes were comparable between β-TM patients and healthy subjects in all the age groups. In the male β-TM population we found a significant effect of age on LVEDVI

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Myocardial fibrosis by late gadolinium enhancement cardiac magnetic resonance and hepatitis C virus infection in thalassemia major patients

Cited by 25 publications

References 32 publications

Cardiac complications in thalassemia major

Cardiac complications in thalassemia major

Cardiac Hemosiderosis in Transfusion DependentThalassemia: A Mini-Review

Biventricular Reference Values by Body Surface Area, Age, and Gender in a Large Cohort of Well‐Treated Thalassemia Major Patients Without Heart Damage Using a Multiparametric CMR Approach

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