Multiparametric Cardiac Magnetic Resonance Survey in Children With Thalassemia Major

Casale, Maddalena; Meloni, Antonella; Filosa, Aldo; Cuccia, Liana; Caruso, Vincenzo; Palazzi, Giovanni; Gamberini, Maria Rita; Pitrolo, Lorella; Putti, Maria Caterina; D’Ascola, Domenico Giuseppe; Casini, Tommaso; Quarta, Antonella; Maggio, Aurelio; Neri, Maria Giovanna; Positano, Vincenzo; Salvatori, Cristina; Toia, Patrizia; Valeri, Gianluca; Midiri, Massimo; Pepe, Alessia

doi:10.1161/circimaging.115.003230

Cited by 66 publications

(47 citation statements)

References 46 publications

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“…The reduced transmission of cardiotrophic viruses might be a possibility, of which one candidate virus is hepatitis C, which has been implicated in myocarditis and cardiac fibrosis [49–51]. Reports of infrequent cardiac fibrosis in beta-thalassemia major using the non-invasive in-vivo technique of late gadolinium enhancement (LGE) CMR showed infrequent minor fibrosis (24%, 2% and 15.8% of patients) in patients without heart failure [52–54], but the extent of fibrosis was limited (3.9%, 0.4%, 1.3%) [52–54]. In comparison to other disease settings it is not clear whether this minor fibrosis would be sufficient to cause significant LV dysfunction, where on average each 1% of infarcted myocardium assessed by LGE leads to only a modest 0.67% reduction in ejection fraction [55].…”

Section: Discussionmentioning

confidence: 99%

Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia

Kirk

Sheppard

Carpenter

et al. 2016

Journal of Cardiovascular Magnetic Resonance

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BackgroundHeart failure related to cardiac siderosis remains a major cause of death in transfusion dependent anaemias. Replacement fibrosis has been reported as causative of heart failure in siderotic cardiomyopathy in historical reports, but these findings do not accord with the reversible nature of siderotic heart failure achievable with intensive iron chelation.MethodsTen whole human hearts (9 beta-thalassemia major, 1 sideroblastic anaemia) were examined for iron loading and fibrosis (replacement and interstitial). Five had died from heart failure, 4 had cardiac transplantation for heart failure, and 1 had no heart failure (death from a stroke). Heart samples iron content was measured using atomic emission spectroscopy. Interstitial fibrosis was quantified by computer using picrosirius red (PSR) staining and expressed as collagen volume fraction (CVF) with normal value for left ventricle <3%.ResultsThe 9 hearts affected by heart failure had severe iron loading with very low T2* of 5.0 ± 2.0 ms (iron concentration 8.5 ± 7.0 mg/g dw) and diffuse granular myocardial iron deposition. In none of the 10 hearts was significant macroscopic replacement fibrosis present. In only 2 hearts was interstitial fibrosis present, but with low CVF: in one patient with no cardiac siderosis (death by stroke, CVF 5.9%) and in a heart failure patient (CVF 2%). In the remaining 8 patients, no interstitial fibrosis was seen despite all having severe cardiac siderosis and heart failure (CVF 1.86% ±0.87%).ConclusionReplacement cardiac fibrosis was not seen in the 9 post-mortem hearts from patients with severe cardiac siderosis and heart failure leading to death or transplantation, which contrasts markedly to historical reports. Minor interstitial fibrosis was also unusual and very limited in extent. These findings accord with the potential for reversibility of heart failure seen in iron overload cardiomyopathy.Trial registrationClinicalTrials.gov Identifier: NCT00520559

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Section: Discussionmentioning

confidence: 99%

Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia

Kirk

Sheppard

Carpenter

et al. 2016

Journal of Cardiovascular Magnetic Resonance

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“…Performing CMRI using gadolinium approach is commonly used for detection of cardiac fibrosis in TDT. 13 Cardiac fibrosis has been associated with lower cardiac T2* value corresponding with greater iron content. 13 However, association of cardiac fibrosis with T2* value may not be always reproducible.…”

Section: Ferritin Levelsmentioning

confidence: 99%

“…13 Cardiac fibrosis has been associated with lower cardiac T2* value corresponding with greater iron content. 13 However, association of cardiac fibrosis with T2* value may not be always reproducible. 38 Kirk et al has reported cases of patients with cardiac fibrosis who had T2* value >20 ms.…”

Section: Ferritin Levelsmentioning

confidence: 99%

“…28 Poor compliance to chelation regimes is a significant factor leading to higher cardiac iron load in TDT patients. 13 Independence of chelation regime, adherence to either of these regimens (DFO, DFX, combined DFO and DFP or DFP alone) when implemented in a proper dose can result in significant improvement in T2* value of the heart. 55 …”

Section: Preventive Measuresmentioning

confidence: 99%

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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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“…7,10,11 It is recommended that the first T2* cardiac magnetic resonance scan should be performed in thalassaemic patients as early as feasible without sedation to tailor the chelation treatment. 12,13 In 2006, the MRI T2* liver and heart technique was introduced at the Sultan Qaboos University Hospital (SQUH), a tertiary care hospital in Muscat, Oman. Initially, only patients over 10 years of age were eligible for the procedure; however, most of the patients failed to complete the procedure due to either movement or an inability to hold their breath in expiration.…”

Section: ‫احلديد‬ ‫زي�دة‬ ‫عن‬ ‫للك�شف‬ ‫أن�شجة‬ ‫لال‬ ‫غريخ�رقة‬ ‫للmentioning

confidence: 99%

Single Breath-Hold Physiotherapy Technique: Effective tool for T2* magnetic resonance imaging in young patients with thalassaemia major

Mevada¹,

Al-Mahruqi²,

El-Beshlawi³

et al. 2016

SQUMJ

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Multiparametric Cardiac Magnetic Resonance Survey in Children With Thalassemia Major

Cited by 66 publications

References 46 publications

Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia

Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia

Cardiac Hemosiderosis in Transfusion DependentThalassemia: A Mini-Review

Single Breath-Hold Physiotherapy Technique: Effective tool for T2* magnetic resonance imaging in young patients with thalassaemia major

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