Multi-Centre Validation of the Cardiovascular Magnetic Resonance Multi Breath-Hold T2* Technique for Myocardial Iron Quantification in Thalassaemia Major.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3828-3828
Author(s):  
Mark A. Tanner ◽  
Taigang He ◽  
Mark A. Westwood ◽  
Renzo Galanello ◽  
Gildo Matta ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Iron Overload ◽  
Breath Hold ◽  
Myocardial Iron ◽  
Thalassaemia Major ◽  
Non Invasive ◽  
Cardiac Siderosis ◽  
Iron Quantification ◽  
Siemens Sonata ◽  
Roll Out

Abstract Introduction: Beta thalassaemia major (TM) is a hereditary anaemia affecting 60 000 births worldwide each year. Survival is dependent upon lifelong blood transfusions resulting in iron overload. Cardiac siderosis can result in a cardiomyopathy which is the leading cause of death in TM. The validated cardiac magnetic resonance (CMR) T2* technique allows non-invasive and reproducible quantification of myocardial iron. Assessment of myocardial iron loading is essential in determining appropriate chelation therapy. This technique has the potential to become the new gold standard in the assessment of cardiac siderosis but is currently available at only a few sites worldwide. For maximal healthcare benefit its inter-scanner reproducibility must be demonstrated before being widely disseminated. Objective: To demonstrate that CMR T2* quantification of myocardial iron can be reproducibly transferred to MR scanners of different manufacturers in different centres. This project was sponsored by the Thalassemia International Federation. Methods: The previously described multi breath-hold gradient echo T2* technique was installed on MR scanners (all 1.5Tesla) at 6 different centres. Scanner details were as follows: Site 1, Phillips Intera (Turin, Italy), Site 2, Siemens Sonata (Philadelphia, USA), Site 3 GE Signa (Limassol, Cyprus) Site 4, Phillips Intera (Nicosia, Cyprus), Site 5, GE Signa (Cagliari, Sardinia) and Philips Intera (Genova, Italy). 34 patients (mean age 30+/− 5.7years) were scanned in total. All patients were subsequently re-scanned at the standardization centre in London, UK (Siemens Sonata, 1.5T) within 31 days of their original scan. Results: The T2* sequence was successfully installed on all 6 scanners. Myocardial T2* values ranged from 3.6ms to 51ms (14.2 +/− 11ms). The overall inter-scanner reproducibility (SD/mean) was 5.3% (figure 1). The mean difference between T2* values at the standardization centre and visited sites was 0.32ms. Conclusion: We have demonstrated that the multi breath-hold T2* technique for the quantification of myocardial iron can be reproducibly transferred to 1.5T MR scanners at different sites and of different manufacturers. There is therefore real potential to roll out this technique worldwide to facilitate maximal healthcare impact in the management of patients with iron overload conditions such as thalassaemia. Figure Figure

Inter-Site Validation of a Single Breath Hold T2* MR Technique for Heart and Liver Iron Measurement.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3830-3830 ◽  
Author(s):  
Paul Kirk ◽  
Lisa J. Anderson ◽  
Mark A. Tanner ◽  
Renzo Galanello ◽  
Gildo Matta ◽  
...  
Keyword(s):  
Coefficient Of Variation ◽  
Breath Hold ◽  
Iron Loading ◽  
Myocardial Iron ◽  
Thalassaemia Major ◽  
Liver Iron ◽  
Single Breath ◽  
Single Breath Hold ◽  
Average Coefficient ◽  
Siemens Sonata

Abstract Background Approximately 60,000 people are born with thalassaemia major every year. The average life expectancy of thalassaemia major patients is 35 years due to iron overload Cardiomyopathy. The cardiomyopathy is reversible when treated early, but once heart failure is established it is often rapidly progressive, and unresponsive to treatment. The single breath hold (SBH) T2* technique has been validated as the most robust and reproducible non-invasive measurement of myocardial and iron load. Our aim in this study was to validate the transferability and reproducibility of this technique in different scanners worldwide. Methods We aim to compare the reproducibility in six different sites worldwide as part of an NIH funded grant (R01-DK66084-01). So far, two of these sites have been validated: Singapore (Siemens Sonata, 1.5T scanner) and Cagliari, Italy (GE Signa, 1.5 T scanner). At both validation sites, 10 patients were scanned for heart and liver T2*, and scans were repeated for interstudy reproducibility. All patients then flew to London to be rescanned on our reference Siemens Sonata scanner. Results Of the 20 patients scanned, 70% had myocardial iron loading (T2* <20ms) and in 10% the myocardial iron loading was severe. Liver iron loading was present in 65% of patients and in 30% this was severe. The coefficient of variation (COV) for the heart T2* measurements between the local sites and London was 5.9% and 4.9% yielding an average coefficient of variation across both sites of 5.4% (figure 1). The coefficient of variation (COV) for the liver T2* measurements between the local sites and London was 11.3% and 3.9% yielding an average coefficient of variation across both sites of 7.6% (figure 2). There was no significant correlation between liver and myocardial loading. Conclusion These are the first data demonstrating the transferability of the SBH T2* technique and the clinical validation from the 2 collaborating centers were excellent for both heart and liver measurements. Further MR sites confirmed for validation include Children’s Hospital of Philadelphia (USA); Ramathibodi Hospital, Bangkok (Thailand); and Chinese University Hong Kong. Figure 1 Figure 1. Figure 2 Figure 2.

Non-Invasive Assessment of Pancreatic Iron Stores by Magnetic Resonance Imaging (MRI) in β-Thalassemic Patients.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4052-4052
Author(s):  
Reijane Alves De Assis ◽  
Andreza Alice Feitosa Ribeiro ◽  
Alexandre Henrique C Parma ◽  
Laércio Alberto Rosemberg ◽  
Cesar Higa Nomura ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Myocardial Iron ◽  
Moderate Correlation ◽  
Transfusion Therapy ◽  
Iron Stores ◽  
Non Invasive ◽  
Cardiac Siderosis ◽  
Liver And Pancreas ◽  
Invasive Method

Abstract Abstract 4052 Poster Board III-987 Background Frequent transfusions of red blood cells are considered standard therapy for patients with β-thalassemia. However, this can lead to transfusional iron overload and subsequent end-organ damage with decrease in life-expectancy. Ferritin is the most widely available non-invasive method for assessing iron stores and iron overload in chronically transfused patients. However, it can also be elevated in inflammatory conditions. MRI has been proposed as a non-invasive method for detection and quantification of iron stores in specific organs. Most studies utilizing MRI for detection of iron overload have focused on the heart and liver, and it is unknown if MRI could satisfactory detect iron overload in other potentially involved organs such as pancreas. Aims To evaluate and correlate the level of iron accumulation in different organs and serum ferritin concentrations for 6 months before imaging studies in patients with β-thalassemia receiving chronic transfusion therapy. Methods MRI was used to asses iron content in three different organs (heart, liver, and pancreas) in patients with a diagnosis of β-thalassemia. Validation of the MRI technique was done by determining liver iron concentration (LIC) from 11 liver biopsies. LIC was determined by atomic absorption spectrometry and was correlated with liver T2* measurement obtained with MRI. There was a significant, curvilinear, inverse correlation between liver T2* MRI measurements and the LIC by Pearson′s method (r =-0.878, p=0.001). We used Pearson′s coefficient of correlation to assess association between T2* measurements among different organs (heart, liver and pancreas) and between organs and serum ferritin levels. Results We evaluated 115 patients with a diagnosis of β-thalassemia that were receiving chronic transfusion therapy. Mean age was 21,25 years (range 7-54 years) and 43% were male. Mean T2* value in the liver was 3.91 ± 3.95 ms, indicating significant liver siderosis (T2*<6.3ms) in most patients (92.1%). Mean value of myocardial T2* was 24.96 ± 14.17 ms and the incidence of cardiac siderosis (T2*<20ms) was 36%. Additionaly, 19% of the patients (22/115) had severe cardiac siderosis (T2* <10ms). Mean T2* value in pancreas was 11.12 ± 11.20 ms, and pancreatic iron deposition (T2* < 21ms) was found in 83.5% of patients. There was no significant correlation between liver and pancreas iron overload (r =0.249), and liver and myocardial iron overload (r =0.149). There was a moderate correlation among pancreas and myocardial iron overload (r =0.546; p=0.001). Mean serum ferritin level was 2,676.5 +/- 2,051.7 ng/mL (range 59-12,362 ng/mL). There was no significant correlation among ferritin serum level and liver, heart and pancreas T2* values (r =-0.397; r =-0.220; r =-0.295). Conclusion Iron overload of liver, heart and pancreas, measured by MRI T2*, could not be predicted by ferritin levels in patients with β-thalassemia. Pancreatic iron overload can be measured by MRI, but we could not predict pancreatic hemosiderosis by detection of iron overload in others organs (except for a moderate correlation among pancreas and heart iron overload). Given that direct calibration of MRI with pancreas biopsies is not possible, further studies are necessary to validate this technique. Disclosures: No relevant conflicts of interest to declare.

Pericardial Effusion Is a Marker of Increased Mortality in Thalassemia Major Patients

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 2689-2689
Author(s):  
Antonella Meloni ◽  
Patrizia Toia ◽  
Leonardo Sardella ◽  
Giuseppe Serra ◽  
Roberta Chiari ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Pericardial Effusion ◽  
Iron Overload ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Myocardial Iron ◽  
Cine Ssfp ◽  
Risk Of Death ◽  
Non Invasive ◽  
Increased Risk

Abstract Introduction. In different types of not-hematological diseases the presence of a small pericardial effusion (PE) was associated with worse survival even after adjustment for patient characteristics, suggesting that it is a marker of underlying disease.In thalassemia major (TM) pericardial effusion was shown to be one of the manifestations of heart disease but its potential prognostic importance has never been investigated in the modern era. Cardiovascular Magnetic Resonance (CMR) by cine SSFP sequences was demonstrated to be extremely sensitive to even a small amount of PE. This is the first prospective study evaluating if the presence of pericardial effusion is associated with increased mortality in TM. Methods. 1259 patients (648 females, mean age 31.02 ± 8.64 years) enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) were prospectively followed from their first Magnetic Resonance Imaging (MRI) scan. CMR was used to quantify myocardial iron (MIO) overload by a multislice multiecho T2* approach and to assess biventricular function parameters and to detect PE by cine SSFP sequences. Results. PE was present in 25 (2.0%) patients.Patients with and without PE were comparable for age and ratio of men/women. At the baseline, the percentage of patients with MIO (global heart T2* value < 20 ms) was comparable between patients with and without PE (12.0 % vs 28.7%; P=0.074) and left ventricular and right ventricular ejection fractions were not significantly different between the two groups. Mean follow-up (FU) time was 44.55 ± 20.35 months and there were 15 deaths. Mortality was greater for patients with PE compared to those without an effusion (8.0% vs 1.1%, P=0.034). PE was a significant predictive factor for death (hazard ratio-HR=12.64, 95%CI=2.78-57.42, P=0.001). PE remained a significant prognosticator for death also in a multivariate model including MIO ms (PE: HR=17.36, 95%CI=3.65-82.62, P<0.0001and global heart T2* < 20 ms: HR=3.07, 95%CI=1.07-8.75, P=0.036). Conclusions. PE is quite rare in TM patients and it is not related to myocardial iron overload. An important role in the development of PE could be played by the 'iron-induced' pericardial siderosis but, due to the limitations of the current non-invasive CMR techniques, we were not able to address this issue. PE was found to be a strong predictor for death, independently by the presence of myocardial iron overload. The non-invasive diagnosis of pericardial effusion is important for a more complete definition of the cardiac involvement of TM patients. The increased risk of death associated with PE may be used along with other clinical characteristics when estimating a patient's prognosis and monitoring. Disclosures Pepe: Chiesi: Speakers Bureau; ApoPharma Inc.: Speakers Bureau; Novartis: Speakers Bureau.

scholarly journals Myocardial iron overload by cardiovascular magnetic resonance native segmental T1 mapping: a sensitive approach that correlates with cardiac complications

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Antonella Meloni ◽  
Nicola Martini ◽  
Vincenzo Positano ◽  
Antonio De Luca ◽  
Laura Pistoia ◽  
...  
Keyword(s):  
Cardiovascular Magnetic Resonance ◽  
Magnetic Resonance ◽  
Iron Overload ◽  
Left Ventricular ◽  
Cardiac Complications ◽  
Lv Function ◽  
Myocardial Iron ◽  
Thalassaemia Major ◽  
Native T1 ◽  
T1 And T2

Abstract Background We compared cardiovascular magnetic resonance segmental native T1 against T2* values for the detection of myocardial iron overload (MIO) in thalassaemia major and we evaluated the clinical correlates of native T1 measurements. Methods We considered 146 patients (87 females, 38.7 ± 11.1 years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassaemia Network. T1 and T2* values were obtained in the 16 left ventricular (LV) segments. LV function parameters were quantified by cine images. Post-contrast late gadolinium enhancement (LGE) and T1 images were acquired. Results 64.1% of segments had normal T2* and T1 values while 10.1% had pathologic T2* and T1 values. In 526 (23.0%) segments, there was a pathologic T1 and a normal T2* value while 65 (2.8%) segments had a pathologic T2* value but a normal T1 and an extracellular volume (ECV) ≥ 25% was detected in 16 of 19 segments where ECV was quantified. Global native T1 was independent from gender or LV function but decreased with increasing age. Patients with replacement myocardial fibrosis had significantly lower native global T1. Patients with cardiac complications had significantly lower native global T1. Conclusions The combined use of both segmental native T1 and T2* values could improve the sensitivity for detecting MIO. Native T1 is associated with cardiac complications in thalassaemia major.

Elevated serum parathormone levels are associated with myocardial iron overload in patients with β-thalassaemia major

2010 ◽  
Vol 84 (1) ◽  
pp. 64-71 ◽  
Author(s):  
Meropi Dimitriadou ◽  
Athanasios Christoforidis ◽  
Marina Economou ◽  
Ioanna Tsatra ◽  
Efthimia Vlachaki ◽  
...  
Keyword(s):  
Iron Overload ◽  
Elevated Serum ◽  
Myocardial Iron ◽  
Thalassaemia Major

Myocardial T2* in Patients with Cardiac Failure Secondary to Iron Overload.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3838-3838 ◽  
Author(s):  
Mark A. Tanner ◽  
John B. Porter ◽  
Mark A. Westwood ◽  
Sunil V. Nair ◽  
Lisa J. Anderson ◽  
...  
Keyword(s):  
Heart Failure ◽  
Iron Overload ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Iron Loading ◽  
Myocardial Iron ◽  
Thalassaemia Major ◽  
Ventricular Ejection Fraction ◽  
Symptomatic Heart Failure ◽  
Increased Risk

Abstract Introduction: Myocardial iron overload is a well established cause of heart failure in a number of haematological disorders and is the leading cause of death in β-thalassaemia major (TM). Once overt heart failure develops prognosis is very poor and it would therefore be desirable to identify patients at risk, prior to the development of symptomatic heart failure. Myocardial iron can now be rapidly and reproducibly assessed using a validated cardiac magnetic resonance (CMR) T2* technique. Left ventricular ejection fraction has been demonstrated to relate to myocardial T2* (normal >20ms), and accordingly iron overloaded patients with symptomatic heart failure are likely to have a low T2* but there are no reports of the myocardial T2* level in newly presenting patients with heart failure and the threshold for increased risk is also unknown. Purpose: To establish the distribution of myocardial T2* values in patients presenting with symptomatic heart failure secondary to iron overload. Methods: Database records of CMR T2* assessments over a 5 year period were reviewed to identify iron overloaded patients presenting with heart failure. Results: 28 patients (median age 29y, 11–79) with iron overload and documented heart failure were identified. 22 patients had thalassaemia major, 3 hereditary haemochromatosis, and 3 had miscellaneous transfusion dependent anaemias. The mean myocardial T2* in all groups was 6.8+/− 2.2ms reflecting severe iron loading by clinical criteria. See figure 1. Myocardial T2* values were similar between sub-groups as follows: TM patients 6.7+/− 2.4ms, haemochromatosis 7.7ms (6.7–7.4ms), miscellaneous 6.8ms (4.8–9.1ms). Conclusion: In this database review, the patients developing heart failure secondary to iron overload all had abnormal myocardial T2*, and overall 89% of heart failure patients had values less than 10ms, which reflects severe myocardial iron loading. This data suggests a myocardial T2* <10ms should be considered a threshold for risk of heart failure (with its attendant high mortality) and such patients should be treated aggressively with increased iron chelation. It also suggests that clinical heart failure occurs nearly exclusively in patients with severe myocardial iron loading. Distribution of myocardial T2+ values in symptomatic heart failure (n=28) Distribution of myocardial T2+ values in symptomatic heart failure (n=28)

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