Cardiac MR images of thalassemia major patients with myocardial iron overload: a data note

Abstract Objective Patients with thalassemia major (TM) have the highest mortality rate due to heart failure induced by myocardial iron overload. However, T2* weighted MR imaging is currently a gold standard approach for measuring iron overload. Examining ventricular volumes with magnetic resonance imaging (MR imaging) and measuring myocardial iron overload in TM patients allows for an early prediction of heart failure. This dataset includes cardiac MR images of TM patients and the control group with clinical and echocardiographic data. This dataset may be useful to researchers investigating myocardial iron overload. This dataset can also be used for medical image processing applications, such as ventricle segmentation. Data description This study provides open-source cardiac MR images of 50 subjects and clinical and echocardiographic data. From February 2016 to January 2019, all images and clinical data were obtained from the MRI department of a general hospital in Mashhad, Iran. All the images are 16-bit gray-scale and stored in DICOM format. All patient-specific information is removed from image headers to preserve patient privacy. In addition, all images associated with each subject are compressed and saved in the RAR format.

Download Full-text

Hypothyroidism and Cardiac Complications In Thalassemia Major Patients

Blood ◽

10.1182/blood.v122.21.2254.2254 ◽

2013 ◽

Vol 122 (21) ◽

pp. 2254-2254 ◽

Cited By ~ 1

Author(s):

Maria Rita Gamberini ◽

Antonella Meloni ◽

Giuseppe Rossi ◽

Giuseppina Secchi ◽

Alfonso D'Ambrosio ◽

...

Keyword(s):

Heart Failure ◽

Heart Disease ◽

Iron Overload ◽

Thalassemia Major ◽

Left Ventricular ◽

Volume Index ◽

Hypothyroid Patient ◽

Myocardial Iron ◽

Heart Dysfunction ◽

Hypothyroid Patients

Abstract Introduction In the non-thalassemic population hypothyroidism has been associated with an increased risk of cardiac disease while the link thyroid-heart disease has been little explored in thalassemia major (TM). This retrospective cohort study aimed to systematically evaluate in a large historical cohort of TM in the cardiovascular magnetic resonance (CMR) era whether hypothyroidism was associated with a higher risk of heart complications (heart failure, arrhythmias and pulmonary hypertension). Methods From a cohort of 957 TM patients who underwent CMR for myocardial iron overload (MIO) assessment, quantification of biventricular function and detection of myocardial fibrosis within the MIOT network (Myocardial Iron Overload in Thalassemia), we identified 115 (12%) hypothyroid patients. Each hypothyroid patient was matched by sex and age (at the time of the CMR) with two non-hypothyroid patients, creating 115 triples. A cardiac event was considered valid if diagnosed at an age older than the hypothyroidism’s onset age for the hypothyroid patient in the belonging triple. Results Hypothyroid and non-hypothyroid patients had comparable MIO, but hypothyroid patients showed significantly lower biventricular stroke volume index, ejection fraction and left ventricular cardiac index. Accordingly, the prevalence of overall heart dysfunction (LV, RV or both) was higher in hypothyroid patients (43.5% vs 33.5%, P=0.0314). Hypothyroid patients had a significant higher frequency of heart failure (19.1% vs 9.1%, P=0.003) and arrhythmias (11.3% vs 4.3%; P=0.003). Figure1 shows odds ratios (OR) estimating the relationship between hypothyroidism and cardiac involvement. Hypothyroid patients had a significant higher risk of heart dysfunction, heart failure and arrhythmias, also adjusting for the endocrine co-morbidity. Conclusions Hypothyroidism seems to increase the risk for heart failure, arrhythmias and heart dysfunction in TM patients. Our data confirm the link thyroid-heart disease also in TM patients and they stress the need to prevent hypothyroidism in this population. Disclosures: No relevant conflicts of interest to declare.

Download Full-text

Left Ventricular Global Function Index and Left Ventricular Mass Volume Ratio By CMR: Relation with Heart Failure in Thalassemia Major Patients

Blood ◽

10.1182/blood.v124.21.2688.2688 ◽

2014 ◽

Vol 124 (21) ◽

pp. 2688-2688

Author(s):

Antonella Meloni ◽

Lucia De Franceschi ◽

Domenico Maddaloni ◽

Sabrina Carollo ◽

Roberto Sarli ◽

...

Keyword(s):

Heart Failure ◽

Iron Overload ◽

Volume Ratio ◽

Thalassemia Major ◽

Left Ventricular ◽

Myocardial Iron ◽

Global Function ◽

Function Index ◽

Lv Mass ◽

Mass Volume

Abstract Introduction: Recently two novels indicators of left ventricular (LV) performance assessed by Cardiovascular Magnetic Resonance (CMR) have been introduced: the LV global function index (LVGFI) and the LV mass/volume ratio (LVMVR). The LVGFI combines LV stroke volume, end-systolic and end diastolic volumes, as well as LV mass, integrating structural as well as mechanical behaviour. Elevated LVMVR is indicative of concentric remodelling. A LVGFI <37% and a LVMVR>1 were shown to be associated with the occurrence of cardiovascular events in no-thalassemic populations. This retrospective cohort study aimed to systematically evaluate in a large historical cohort of thalassemia major (TM) in the CMR era whether the LVGFI and the LVMVR were associated with a higher risk of heart failure. Methods: We considered 812 TM patients (391 M, 30.4±8.6 years), consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network. LVGFI and LVMRI were quantitatively evaluated by SSFP cine images. The T2* value in all the 16 cardiac segments was evaluated and a global heart T2* value <20 ms was considered indicative of myocardial iron overload (MIO). Results: Eighty (9.9%) patients had a LVGFI<37% and, compared to the patients with a normal LVGFI, they showed a significant higher frequency of heart failure (43.8% vs 4.2%; P<0.0001). Patients with a LVGFI<37% had a significant higher risk of heart failure (odds-ratio-OR=17.59, 95%CI=9.95-21.09; P=<0.001). The risk remained significant also adjusting for the presence of MIO (OR=15.54, 95%CI=8.05-26.27; P=<0.001). Thirty (3.7%) patients had a LVMVR≥1% and, compared to the patients with a normal LVMRI, they showed a significant higher frequency of heart failure (20.0% vs 7.7%; P=0.015). Patients with a LVMVR≥1% had a significant higher risk of heart failure (OR=3.01, 95%CI=1.18-7.64; P=0.021). The risk remained significant also adjusting for the presence of MIO (OR=3.44, 95%CI=1.31-9.01; P=0.012). In a multivariate model including LVGFI, LVMVR and heart iron, the significant predictors of heart failure were a LVGFI<37% (OR=14.05, 95%CI=7.66-25.77; P=<0.001) and a global heart T2*<20 ms (OR=1.94, 95%CI=1.08-3.47; P=0.026). Conclusions: In TM patients a LVGFI<37% was associated with an higher risk of heart failure, independent by the presence of MIO. A widespread program using CMR exploiting its multi-parametric potential can have considerable power for the early identification and treatment of patients at risk for heart failure. Disclosures No relevant conflicts of interest to declare.

Download Full-text

Efficacy and Safety of Amlodipine in Preventing Myocardial Iron Overload in Patients with Transfusion-Dependent Thalassemia Major : A Systematic Review and Meta-Analysis

Blood ◽

10.1182/blood-2020-137713 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 48-49

Author(s):

Richi Kashyap ◽

Muhammad Ashar Ali ◽

Saad Ullah Malik ◽

Farhan Khalid ◽

Ali Jaan ◽

...

Keyword(s):

Adverse Effects ◽

Iron Overload ◽

Meta Analysis ◽

Iron Concentration ◽

Iron Chelation ◽

Thalassemia Major ◽

Iron Toxicity ◽

Control Group ◽

P Value ◽

Myocardial Iron

Background: Patients diagnosed with thalassemia major who are transfusion dependant, have iron accumulation leading to iron toxicity and severe impairment in organs like heart, liver and endocrine organs which are highly sensitive to iron toxicity. This makes iron chelation therapy imperative for these patients. Half of the deaths resulting from iron toxicity related complications are attributed to cardiac complications. Iron chelation therapies have not been completely successful to prevent iron toxicity related complications like arrhythmia, cardiomyopathy and heart failure. Higher doses of iron chelation therapies have been associated with various side effects. Studies have shown L-type calcium channel blocker might be able to reduce iron uptake by myocardium. The aim of this meta-analysis is to assess the efficacy and safety of amlodipine to reduce myocardial iron concentration (MIC). Methods: We used PICO framework to do a systematic literature search using four database PubMed, Cochrane, Embase, and Web of Science using keywords, "Thalassemia" AND "Amlodipine" from the inception till July 2020. The initial search showed 90 articles out of which, six randomized clinical trials (RCT) (N= 226) were selected after exclusion of case reports, case series, preclinical trials, review articles, meta-analysis, and trials not providing any information about preventing iron overload in patients with transfusion dependent thalassemia. We extracted the data for myocardial iron concentration (MIC), myocardial T2, ferritin, hepatic iron/liver iron concentration (LIC), liver T2, left ventricular ejection fraction, response rate and adverse effects. DerSimonian-Laird random effects model was used to derive mean differences along with their 95% confidence interval (CI) using comprehensive meta-analysis version 3.0. Results: In six RCT, 96 patients were tested in experimental group and 97 in control group. In five RCT total number of male participants were 45 in experimental group and 54 in control group. 33 patients had splenectomy in experimental group and 41 in control group. The age range was 8 years to 31 years. The myocardial T2 score increased in amlodipine group compared to standard chelation group with significant mean difference estimated to be -0.62 (95% CI: -0.95-0.29, p-value: <0.001) in favor of amlodipine in meta-analysis of the four trials (Fig 1.). Statistically significant reduction in myocardial iron was seen in two trials on adding amlodipine to standard chelation therapy (N=55) (Table 1.) (Khaled et al and Fernandes et al). Significant difference was reported in liver T2 score and LIC at the end of six months between amlodipine and control group by Khaled et al. But, there was no statistically significant mean difference in serum ferritin and in liver MRI T2 between amlodipine group and control group with mean difference of -1143 (95% CI: -2410 to 124, p-value = 0.07) and -0.06 (95% CI: -0.463 to 0.338, p-value = 0.76) in meta-analysis of four and two trials respectively (Fig 2. And Fig 3.). El-Haggar et al compared amlodipine with spirulina and statistically significant improvement in myocardial T2 and NT-proBNP level was seen in both groups. Spirulina group also showed significant reduction in serum ferritin, which showed spirulina could also help reduce iron overload. Only mild adverse effects were reported by trials (Table 2.). No cases of severe hypotension, palpitation or any other serious adverse effects were seen in the amlodipine group. Conclusion: This systematic review and meta-analysis suggests that addition of amlodipine 2.5-5 mg/day to standard chelation therapy with monitoring for potential adverse effects, could benefit patients with thalassemia major by reducing cardiac iron overload and thus improve survival and quality of life. Future studies are required to study the role of amlodipine in reducing iron overload in endocrine organs that also absorb iron through voltage-gated channels, particularly considering the close association of cardiac siderosis with endocrine complications and the correlation of pancreas and MICs. Disclosures Anwer: Incyte, Seattle Genetics, Acetylon Pharmaceuticals, AbbVie Pharma, Astellas Pharma, Celegene, Millennium Pharmaceuticals.: Honoraria, Research Funding, Speakers Bureau.

Download Full-text

4326Reduction of cardiac outcomes in thalassemia major thanks to a ten-year national Italian networking

European Heart Journal ◽

10.1093/eurheartj/ehz745.0163 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

A Meloni ◽

L Pistoia ◽

N Giunta ◽

N Schicchi ◽

P Giuliano ◽

...

Keyword(s):

Heart Failure ◽

Iron Overload ◽

Cause Of Death ◽

Chelation Therapy ◽

Thalassemia Major ◽

Cardiac Complications ◽

Myocardial Iron ◽

Cardiac Iron ◽

Cardiac Outcomes ◽

Number Of Patients

Abstract Introduction The MIOT (Myocardial Iron Overload in Thalassemia) Network was a network of thalassemia and CMR centers built in 2006 in order to assure homogeneous and standardized cardiac iron overload assessment for a significant number of patients. Purpose We describe the impact of this ten-year Network on cardiac iron, complications and deaths in patients with thalassemia major (TM). Methods 1746 TM patients (911 F; age 31.17±9.09 yrs) were enrolled in the MIOT Network. Myocardial iron overload (MIO) was quantified by the multislice multiecho T2* technique. Biventricular function was quantified by cine images. Results 1392 TM patients performed an end-of-study CMR. At the last CMR significantly higher global heart T2* values (35.44±10.69 ms vs 29.16±12.02 ms; P<0.0001) and a significant lower number of patients with global heart T2*<20 ms (26.3% vs 12.0%; P<0.0001) were detected. Four patterns of MIO were identified: no MIO (all segments with T2*≥20 ms), heterogeneous MIO and global heart T2*≥20 ms, heterogeneous MIO and global heart T2*<20 ms, and homogeneous MIO (all T2*<20 ms). At the last CMR a significant higher frequency of patients with no MIO and a significant lower frequency for the other three patterns indicating MIO were found (Figure 1). In patients with global heart T2*<20 ms a significant increase in left ventricular ejection fraction (EF) (difference: 3.2±8.5%, P<0.0001) as well as in right ventricular EF (difference: 1.2±8.9%, P=0.002) were detected. Based on CMR results the 75% of the patients changed the chelation therapy. At the last CMR the percentage of patients with an excellent/good compliance was significantly higher (94.8% vs 92.2%%; P<0.0001). The complete history of cardiac complications-CC (heart failure, arrhythmias, pulmonary hypertension, myocardial infarction, angina, myo/pericarditis, peripheral vascular disease) was present for 1062 patients. Out of the 1001 patients with resolved CC or without CC before the enrolment in the project, the 6.6% had a CC before the enrolment in the project. During the study, the frequency of CC was 4.4%, significantly lower (P=0.023). In particular, the frequency of heart failure (HF) was significantly lower (3.5% vs 0.8%, P<0.0001). Forty-six patients died during the study. HF continues to be the leading cause of death (30.4% of all causes), but there was a consistent decline in HF mortality rate, that was 60.2% in an Italian study dated 2004. No patients died for arrhythmias while cancer was the second leading cause of death. Conclusion Over a period of 10 years, the continuous monitoring of cardiac iron levels and a tailored chelation therapy allowed a reduction of MIO in the 70% of patients, with consequent improvement of cardiac function and reduction of cardiac complications and mortality from MIO-related HF. So, a national networking was effective in improving the care and reducing cardiac outcomes of TM patients.

Download Full-text

Diabetes Mellitus and Cardiac Complications in Thalassemia Major Patients

Blood ◽

10.1182/blood.v118.21.2150.2150 ◽

2011 ◽

Vol 118 (21) ◽

pp. 2150-2150

Author(s):

Maria Rita Gamberini ◽

Antonella Meloni ◽

Vincenzo Caruso ◽

Marcello Capra ◽

Paolo Cianciulli ◽

...

Keyword(s):

Diabetes Mellitus ◽

Heart Failure ◽

Iron Overload ◽

Myocardial Fibrosis ◽

Thalassemia Major ◽

Cardiac Complication ◽

Cardiac Complications ◽

Myocardial Iron ◽

Heart Dysfunction ◽

Co Morbidity

Abstract Abstract 2150 Introduction. In thalassemia major (TM) patients myocardial iron overload and chronic anemia are the recognized leading causes of cardiomyopathy, but a role can also be played by other factors such as endocrine abnormalities. The aim of this retrospective study was to evaluate if diabetes mellitus (DM) was associated with an higher prevalence and risk of cardiac dysfunction and of heart complications, regardless to the presence of myocardial iron overload. Methods. From a cohort of 957 TM patients who underwent MRI within the MIOT network (Myocardial Iron Overload in Thalassemia), among the patients (N = 358) with no cardiac iron (all cardiac segments with a T2* ≥ 20 ms) we identified 29 patients with DM and 329 patients without DM. The normal values of ejection fraction (EF) normalized by sex and age, obtained in a cohort of 142 TM patients without cardiac disease and iron overload, were used to define left ventricular (LV) and right ventricular (RV) heart dysfunction (EF < mean – 2 standard deviation). Heart failure (HF) was diagnosed by Magnetic Resonance Imaging (MRI) in presence of a LV and/or RV EF lower than 4 standard deviations from the normalized mean value and by a positive history (clinical symptoms, confirmation by physical examination and treatment). Myocardial fibrosis was evaluated by delayed enhancement MRI technique. Results. The prevalence of overall heart dysfunction (LV, RV or both) was higher in patients with DM (44.8%) versus patients without DM (28.3%), with a P-value very close to the statistically significance (P=0.061). In more details, patients with DM presented significantly more biventricular dysfunction (20.7% vs 7.6%, P=0.016). The prevalence of myocardial fibrosis was significantly higher in the DM patients vs the no DM patients (37.5% vs 19.2 %; P=0.033). Cardiac complications occurred with a significantly higher frequency in patients with DM (55.2% vs 15.5%, P<0.0001). Taking into account each cardiac complication separately, a significant difference between the groups was found in the occurrence of heart failure (27.6% vs 9.4%, P<0.003) and hyperkinetic arrhythmias (34.5% vs 5.2%,P<0.0001), both supraventricular (27.6% vs 4%, P<0.0001) and ventricular (6.9% vs 0.6%, P=0.034). Table 1 shows odds ratios (OR) estimating the relationship between diabetes and cardiac involvement. Among cardiac dysfunctions, only the biventricular forms were significantly positively associated with the diabetes. However, the correction for age caused the loss of the significance. The association between DM and myocardial fibrosis became not significant after the correction for age and endocrine co-morbidity. Patients with DM were significantly more likely to have cardiac complications and the results were not affected by the adjustment for age and/or endocrine co-morbidity. Considering separately each cardiac complication, a significant association was found for HF and hyperkinetic arrhythmias. The association between DM and HF resulted not significant after the correction for age. For the hyperkinetic arrhythmias, the OR remained significant after the correction for age and/or endocrine co-morbidity. Conclusion. In TM patients without myocardial iron DM was significantly associated with the presence of cardiac complications globally considered and hyperkinetic arrhythmias. Disclosures: No relevant conflicts of interest to declare.

Download Full-text

Gender Differences in the Development of CMR Abnormalities and Cardiac Complications: A Multicentric Prospective Study in a Large Cohort of Thalassemia Major Patients

Blood ◽

10.1182/blood.v128.22.849.849 ◽

2016 ◽

Vol 128 (22) ◽

pp. 849-849 ◽

Cited By ~ 1

Author(s):

Alessia Pepe ◽

Silvia Maffei ◽

Laura Pistoia ◽

Angela Ciancio ◽

Giovanni Palazzi ◽

...

Keyword(s):

Heart Failure ◽

Iron Overload ◽

Myocardial Fibrosis ◽

Ventricular Dysfunction ◽

Thalassemia Major ◽

Cardiac Complications ◽

Myocardial Iron ◽

Cardiac Iron ◽

Cardiac Evaluation

Abstract Introduction. We aimed to prospectively assess if the male gender was associated with an higher risk of progressive cardiac iron accumulation, development of biventricular dysfunction and myocardial fibrosis assessed by CMR, and development of cardiac complications including heart failure (HF), arrhythmias and pulmonary hypertension (PH). Methods. We considered 1711 TM patients (899 females, 31.09±9.08 years), consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network. Myocardial iron overload was assessed by the multislice multiecho T2* technique. Biventricular function was quantified by cine images. Late gadolinium enhancement (LGE) images were acquired to detect myocardial fibrosis. Results. Although having a similar risk of accumulating iron, males showed a significant higher risk of developing cardiac dysfunction, heart failure, arrhythmias and cardiac complications globally considered (Table 1). Figure 1 shows the Kaplan-Meier curves for the outcomes for which the male sex was a significant prognosticator. Until 20-30 years of follow-up the two lines (male and female sex) were almost overlapping while after they clearly diverged. So, patients were divided in two groups based on the follow-up duration. A significant gender-specific difference in the frequency of ventricular dysfunction and cardiac complications appeared for patients followed for at least 20 years. So, two subgroups of patients were identified: patients followed for less than 20 years and patients followed for more than 20 years. In the first subgroup males and females had a comparable risk of developing cardiac iron overload, ventricular dysfunction and cardiac complications. Conversely, if a follow-up longer than 20 years was considered, males exhibited a significant higher risk of having ventricular dysfunction, heart failure, arrhythmias, and cardiac complications. Conclusion. Females seem to tolerate iron toxicity better, possibly as an effect of reduced sensitivity to chronic oxidative stress. According to the International Guidelines, TM patients should perform a complete cardiac evaluation every year. Our study suggested that in females older than 20 years the follow-up may be performed every 24 months, thus reducing health care costs. Table 1 Table 1. Figure 1 Figure 1. Disclosures Pepe: Chiesi Farmaceutici and ApoPharma Inc.: Other: Alessia Pepe is the PI of the MIOT project, that receives no profit support from Chiesi Farmaceutici S.p.A. and ApoPharma Inc.. De Franceschi:F. Hoffmann-La Roche Ltd, Basel, Switzerland: Research Funding.

Download Full-text

Impact of a Ten-Year National Italian Networking on Cardiac Complications in Patients with Thalassemia Major

Blood ◽

10.1182/blood-2018-99-112546 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 1055-1055

Author(s):

Alessia Pepe ◽

Laura Pistoia ◽

Aurelio Maggio ◽

Annamaria Carrà ◽

Maria Grazia Roberti ◽

...

Keyword(s):

Heart Failure ◽

Iron Overload ◽

Chelation Therapy ◽

Thalassemia Major ◽

Left Ventricular ◽

Mean Difference ◽

Left Ventricular Ejection ◽

Cardiac Complications ◽

Myocardial Iron ◽

Number Of Patients

Abstract Introduction. The MIOT (Myocardial Iron Overload in Thalassemia) Network was a national Italian network constituted by thalassemia and magnetic resonance imaging (MRI) centers built in 2006. The main aim was to assure available, accessible homogeneous and standardized T2* MRI cardiac and liver iron overload assessments for a significant number of patients with emoglobinopathies. Moreover, the creation of a solid clinical-instrumental web based database allowed data exchange between centers and constituted a means of monitoring health care processes and outcomes. We describe the impact of this ten-year Network on cardiac complications in patients with thalassemia major (TM). Methods. Among the 2497 emoglobinopathies patients consecutively enrolled in the MIOT Network we considered the 1401 TM patients who performed an end-of-study MRI. Per protocol the MRI follow up was scheduled every 18±3 months. Myocardial iron overload (MIO) was quantified by the multislice multiecho T2* technique. Biventricular function was quantified by cine images. Results. At the last MRI significantly higher global heart T2* values (35.5±10.7 ms vs 29.2±12.0 ms; P<0.0001) and a significant lower number of patients with global heart T2*<20 ms (26.3% vs 12.0%; P<0.0001) were detected. Four patterns of MIO were identified: no MIO (all segments with T2*≥20 ms), heterogeneous MIO and global heart T2*≥20 ms, heterogeneous MIO and global heart T2*<20 ms, and homogeneous MIO (all segments with T2*<20 ms). Figure 1 shows the frequency of the 4 patterns at both scans. At the last MRI a significant higher frequency of patients with no MIO and a significant lower frequency for the other three patterns indicating MIO were detected. An improvement in MIO, that is a transition to a better risk class, was detected in the 68.4% of patients showing MIO at the baseline (at least one pathologic segment). In patients with significant baseline MIO (global heart T2*<20 ms) a significant increase in left ventricular ejection fraction (EF) (mean difference: 3.2±8.5 %, P<0.0001) as well as in right ventricular EF (mean difference: 1.2±8.9 %, P=0.002) were detected with a concordant improvement of MIO status. Based on MRI results the 75% of the patients changed the chelation therapy. At the last MRI the percentage of patients with an excellent/good compliance was significantly higher (94.7% vs 92.7%%; P=0.034). The 13.1% of the patients had a cardiac complication (heart failure, arrhythmias, pulmonary hypertension, myocardial infarction, angina, myo/pericarditis, and peripheral vascular disease) before the enrolment in the project. During the study, the frequency of cardiac complications was 7.9 %, significantly lower (P<0.0001). In particular, the frequency of heart failure was significantly lower (5.9% vs 1.7%, P<0.0001). Conclusion. Over a period of 10 years, the continuous monitoring of cardiac iron levels and a tailored chelation therapy allowed a reduction of MIO in the 70% of patients and a consequent improvement of cardiac function and reduction of heart failure. So, a national networking as the MIOT project was effective in improving the care and reducing cardiac outcomes of TM patients. Figure 1 Figure 1. Disclosures Pepe: Chiesi Farmaceutici S.p.A., ApoPharma Inc., and Bayer: Other: No profit support.

Download Full-text

P601Heart disease and pancreatic iron in thalassemia major

European Heart Journal ◽

10.1093/eurheartj/ehz747.0210 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

A Meloni ◽

L Pistoia ◽

G Restaino ◽

S Renne ◽

N Giunta ◽

...

Keyword(s):

Heart Failure ◽

Heart Disease ◽

Iron Overload ◽

Myocardial Fibrosis ◽

Strong Predictor ◽

Pancreatic Head ◽

Thalassemia Major ◽

Cardiac Complications ◽

Myocardial Iron ◽

Cardiac Iron

Abstract Background Some preliminary data have postulated a correlation between pancreatic iron overload and heart iron and function in thalassemia major (TM) patients. Purpose In the present multicenter study we explored systematically the link between pancreatic iron and heart disease in a large cohort of TM patients. Methods We considered 880 TM patients (467 M, mean age 37.83±10.05 years) enrolled in the E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) project. T2* measurements were performed over pancreatic head, body and tail and global value was the mean. Myocardial iron overload (MIO) was quantified using a T2* segmental approach. Biventricular function parameters were assessed by cine images. Late gadolinium enhancement (LGE) images were acquired to detect myocardial fibrosis. Results A significant correlation between pancreatic and cardiac iron was reconfirmed in this more numerous population and a normal pancreas T2* showed negative predictive value of 100% for cardiac iron. LGE sequences were acquired in 273 TM patients and 84 (30.77%) of them showed macroscopic myocardial fibrosis. Global pancreas T2* values were significantly lower in patients with fibrosis (7.38±6.19 ms vs 11.91±9.79 ms; P<0.0001). Sixty-four patients had at least one cardiac complication (arrhythmias, heart failure, pulmonary hyperthension, vascular disease). Patients with cardiac complications showed a significant lower global pancreas T2* (7.83±5.61 ms vs 12.76±10.34 ms; P=0.024). Specifically, global pancreas T2* values were significantly lower in patients with heart failure as well as in patients with arrhythmias and all patients with one of these two diseases had a pathologic global pancreas T2* value (see Figure). Conclusion Pancreatic iron is a strong predictor not only for cardiac iron, but also for cardiac complications supporting a more profound link between pancreatic iron and heart disease in TM.

Download Full-text

Different Patterns of Myocardial Iron Overload by T2* Cardiovascular MR As Markers of Risk for Cardiac Complication in Thalassemia Major,

Blood ◽

10.1182/blood.v118.21.3190.3190 ◽

2011 ◽

Vol 118 (21) ◽

pp. 3190-3190

Author(s):

Antonella Meloni ◽

Pasquale Pepe ◽

Petra Keilberg ◽

Brunella Favilli ◽

Vincenzo Positano ◽

...

Keyword(s):

Heart Failure ◽

Iron Overload ◽

Odds Ratio ◽

Chelation Therapy ◽

Conflicts Of Interest ◽

Basal Medium ◽

Thalassemia Major ◽

Cardiac Complications ◽

Myocardial Iron ◽

Group I

Abstract Abstract 3190 Introduction: Cardiac complications mainly related to myocardial iron overload (MIO) remain the main cause of morbidity and mortality in thalassemia major (TM). Thalassemia cardiomyopathy is treatable and partly reversible if appropriate chelation therapy is instituted in time. The validated multislice multiecho T2* Cardiovascular Magnetic Resonance (CMR) technique has permitted to quantify segmental and global myocardial iron burden detecting different patterns of iron overload. The aim of this study was to verify the risk of cardiac complications related to different patterns of MIO in a large cohort of TM patients. Methods: We considered 812 TM patients for who CMR and cardiac data were collected in a central data base. Three short-axis views (basal, medium, apical) of the left ventricle were acquired using a multislice multiecho T2* sequence. Using a previously validated software the 16 segmental T2* values and the mean global heart T2* value were provided. A conservative cut off of 20 ms was considered the limit of normal for the segmental and global T2* values. Results: We identified 4 groups of patients: group I (17%) with homogeneous MIO (all segments with T2*<20 ms), group II (12%) with heterogeneous MIO (some segments with T2*<20 ms and others with T2*≥20 ms) and global heart T2*<20 ms; group III (29%) with heterogeneous MIO and global heart T2*≥20 ms; group IV (42%) with no MIO (all segments with T2*≥20 ms). The clinically relevant findings in the 4 groups are summarized in Table 1. The percentage of patients with cardiac complications was significantly different in the 4 groups (P<0.0001). In particular, the percentage of patients with heart failure was significantly different in the 4 groups (P<0.0001). No significant differences were found among groups in the percentage of arrhythmias and pulmonary hypertension. Odds Ratio for cardiac complications was 1.7 (1.0–2.7 OR 95% CI; P=0.041) for patients with homogeneous MIO versus patients with no MIO. Odds Ratio for heart failure was 2.3 (1.3–4.2 OR 95% CI; P=0.004) for patients with homogeneous MIO versus patients with no MIO and 2.2 (1.1–4.2 OR 95% CI; P=0.020) for patients with heterogeneous MIO and global heart T2*<20 ms versus patients with no MIO. Conclusions: Homogeneous MIO predicts a significantly higher risk to develop cardiac complications, especially heart failure, suggesting an intensive chelation therapy in this group of patients. Disclosures: No relevant conflicts of interest to declare.

Download Full-text