Role of CMR feature-tracking derived left ventricular strain in predicting myocardial iron overload and assessing myocardial contractile dysfunction in patients with thalassemia major

2021 ◽  
Author(s):  
Vineeta Ojha ◽  
Kartik P. Ganga ◽  
Tulika Seth ◽  
Ambuj Roy ◽  
Nitish Naik ◽  
...  
Keyword(s):  
Iron Overload ◽  
Feature Tracking ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Contractile Dysfunction ◽  
Myocardial Iron ◽  
Left Ventricular Strain ◽  
Myocardial Contractile

scholarly journals Association between Native Myocardial T1 Mapping and Cardiac Function and Macroscopic Fibrosis in Thalassemia Major

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 26-27
Author(s):  
Alessia Pepe ◽  
Nicola Martini ◽  
Antonio De Luca ◽  
Vincenzo Positano ◽  
Laura Pistoia ◽  
...  
Keyword(s):  
Iron Overload ◽  
Myocardial Fibrosis ◽  
T1 Mapping ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Lv Function ◽  
Myocardial Iron ◽  
Native T1 ◽  
Molli Sequence ◽  
Lv Volume

Background.Cardiovascular magnetic resonance (CMR) is the only available technique for the non-invasive quantification of MIO. The native T1 mapping has recently been proposed as an alternative to the universally adopted T2* technique, due to the higher sensitivity for detection of changes associated with mild or early iron overload. Objective.To study the association between T1 values and left ventricular (LV) function in thalassemia major (TM) and to evaluate for the first time if T1 measurements quantifying MIO are influenced by macroscopic myocardial fibrosis. Methods.146 TM patients (87 females, 38.7±11.1 years) consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia Network underwent CMR. Native T1 values were obtained by Modified Look-Locker Inversion recovery (MOLLI) sequence in all 16 myocardial segments and the global value was the mean. LV function parameters were quantified by cine images. Late gadolinium enhancement (LGE) technique was used to detect macroscopic myocardial fibrosis. Results.No correlation was detected between global heart T1 values and LV volume indexes, LV mass index, or LV ejection fraction. Foourteen (9.6%) patients had an abnormal LV motion (13 hypokinesia and 1 dyskinesia) and they showed significantly lower global heart T1 values than patients without LV motion abnormalities (883.8±139.7 ms vs 959.0±91.3 ms; P=0.049). LGE images were acquired in 88 patients (60.3%) and macroscopic myocardial fibrosis was detected in 36 patients (40.9%). The 72.2% of patients had two or more foci of fibrosis. Patients with macroscopic myocardial fibrosis had significantly lower global heart T1 values (921.3±100.3 ms vs 974.5±72.7 ms; P=0.027) (Figure 1A). Data about the LGE was present for 1408 segments (88 patients x 16 segments) and 105 (7.5%) were positive. Segments with LGE had significantly lower T1 values than segments LGE-negative (905.6±110.6 ms vs 956.9±103.8 ms; P<0.0001) (Figure 1B). Conclusion.No correlation between T1 values and LV function parameters was detected, probably because the majority of the patients had normal or mild abnormal LV parameters. TM patients with macroscopic myocardial fibrosis showed significantly lower T1 values suggesting that T1 measurements for quantifying MIO are not influenced by macroscopic myocardial fibrosis and an association between myocardial iron and macroscopic fibrosis, previously detected only in pediatric TM patients. Figure Disclosures Pepe: Chiesi Farmaceutici S.p.A.:Other: no profit support and speakers' honoraria;Bayer:Other: no profit support;ApoPharma Inc.:Other: no profit support.Pistoia:Chiesi Farmaceutici S.p.A.:Other: speakers' honoraria.Meloni:Chiesi Farmaceutici S.p.A.:Other: speakers' honoraria.

Prospective Survey on Heart and Liver Iron and Heart Function in Thalassemia Major Patients Treated with Sequential deferiprone–desferrioxamine Versus Deferiprone and Desferrioxamine in Monotherapy

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5298-5298
Author(s):  
Alessia Pepe ◽  
Antonella Meloni ◽  
Giuseppe Rossi ◽  
Anna Spasiano ◽  
Domenico Giuseppe D'Ascola ◽  
...  
Keyword(s):  
Cardiac Function ◽  
Iron Overload ◽  
Heart Function ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
The Other ◽  
Hepatic Iron ◽  
Myocardial Iron ◽  
Liver Iron ◽  
Biventricular Function

Abstract Abstract 5298 Introduction: Magnetic Resonance (MR) is the unique non invasive suitable technique to evaluate quantitatively the changes in cardiac and hepatic iron and in cardiac function in thalassemia major (TM) patients under different chelation regimens. This study aimed to prospectively assess the efficacy of the sequential deferiprone–deferrioxamine (DFP-DFO) versus deferiprone (DFP) and deferrioxamine (DFO) in monotherapy in a large cohort of TM patients by quantitative MR. Methods: Among the first 1135 TM patients enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network, 392 patients performed a MR follow up study at 18±3 months. We evaluated prospectively the 35 patients treated with DFP-DFO versus the 39 patients treated with DFP and the 74 patients treated with DFO between the 2 MR scans. Iron concentrations were measured by T2* multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images. Results: Excellent/good levels of compliance were similar in the DFP-DFO (97.1%) versus DFP (94.9%) and DFO (95.9%) groups. No significant differences were found in the frequency of side effects in DFP-DFO (15.6%) versus DFP group (9.4%). The percentage of patients who maintained a normal global heart T2* value (≥20 ms) was comparable between DFP-DFO (96%) versus DFP (100%) and DFO (98.1%) groups. Among the patients with myocardial iron overload (MIO) at baseline (global heart T2*<20 ms), in all three groups there was a significant improvement in the global heart T2* value (DFO-DFP: P=0.004, DFP: P=0.015 and DFO: ms P=0.007) and a significant reduction in the number of pathological segments (DFO-DFP: P=0.026, DFP: P=0.012 and DFO: P=0.002). In DFO-DFP and DFP groups there was a significant increment in the left ventricular (LV) ejection fraction (EF) (P=0.035 and P=0.045, respectively) as well as in the right ventricular (RV) EF (P=0.017 and P=0.001, respectively). The improvement in the global heart T2* and in biventricular function were not significantly different in DFO-DFP compared to the other groups (Table 1). Among the patients with hepatic iron at baseline (T2*<9.2 ms), only in DFO group there was a significant improvement in the liver T2* value (2.0±3.5 ms P=0.010). Liver T2*changes were not significantly different in DFO-DFP versus the other groups. Conclusions: Prospectively we did not find significant differences on cardiac and hepatic iron or in cardiac function in TM patients treated with sequential DFP–DFO therapy versus the TM patients treated with DFO or DFP in monotherapy. Disclosures: Pepe: Novartis: Speakers Bureau; Apotex: Speakers Bureau; Chiesi: Speakers Bureau. Off Label Use: Association of two chelators commercially available in order to obtain a higher efficacy. Lai:Novartis: Honoraria, Research Funding.

Hypothyroidism and Cardiac Complications In Thalassemia Major Patients

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2254-2254 ◽  
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.

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

Blood ◽  
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.

Gender Differences in Iron Overload and Left Ventricular Function in a Cohort of Thalassemia Major Patients: A Prospective Study of a Single Italian Center Using Cardiovascular Magnetic Resonance.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3819-3819
Author(s):  
Elisabetta Volpato ◽  
Elena Cassinerio ◽  
Maria Rosaria Fasulo ◽  
Paola Pedrotti ◽  
Stefano Pedretti ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Gender Differences ◽  
Cardiovascular Magnetic Resonance ◽  
Iron Overload ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Myocardial Iron ◽  
Iron Intake ◽  
Cardiac Iron ◽  
Cardiac Iron Overload

Abstract Introduction: cardiac failure due to secondary iron overload remains the main cause of death in patients with b-Thalassemia Major (TM). Cardiovascular Magnetic Resonance Imaging (CMR) T2* technique is a new tool to assess myocardial iron concentration that allows to tailor the optimal iron chelation treatment for each patient. Aim of the study: to assess left ventricular function and myocardial iron overload in a cohort of TM patients, cared at Hereditary Anemia Center in Milan, Italy. Methods and Results: In 91 TM patients (33 males/58 females, mean age 32 ± 6 yrs) myocardial iron loading was assessed with the use of CMR T2* measurements (CMR Tools, Cardiovascular Imaging Solutions, London, UK). Left ventricular ejection fraction (LVEF) was also assessed with CMR. In the overall group hemoglobin levels were 9.0 ± 1.0 g/dl; the mean serum ferritin levels and iron intake during the six months before CMR evaluation were 1507 ± 1884 ng/ml and 0.34 ± 0.08 mg/kg/die respectively. T2* was significantly different between females and males (24 ± 11 and 32 ± 12 ms, respectively; p &lt; 0.0001), with significant differences in diabetes mellitus prevalence (17% vs 8%, p&lt;0.01) but not in age, serum ferritin, iron intake and hemoglobin levels (Table 1). Seven (7.6%) asymptomatic females showed a severe cardiac iron overload (T2* ≤ 10 ms), 9 patients (9.9%) moderate (T2* between 10.1 and 14 ms), 15 patients (16.4%) mild cardiac iron overload (T2* between 14.1 and 20 ms) and 60 patients (65.9%) had normal T2* (&gt; 20 ms). LVEF was significantly different between females and males (35% vs 57%, p&lt;0.001) with evidence of a significant relationship between iron overload severity and LVEF impairment (r=0.92). Conclusions: CMR cardiac function and T2* assessment allow to detect pre-symptomatic cardiac iron overload. Females are more at risk for severe iron overload and left ventricular impairment. The prevalence of diabetes mellitus and compliance to chelation therapy could be relevant in explaining the gender differences. Clinical parameters and T2* values in men and women with thalassemia major Men p Women SD: standard deviation Number of patients (n. of pts) 33 - 58 Age ± SD (years) 33 ± 6 ns 32 ± 6 Hemoglobin levels ± SD (g/dl) 9.0 ± 1.7 ns 9.0 ± 0.8 Ferritin levels ± SD (ng/ml) 964 ± 891 ns 1821 ± 2216 Iron intake ± SD (mg/Kg/die) 0.30 ± 0.07 ns 0.36 ± 0.09 Mean T2* value ± SD (ms) 32 ± 12 &lt;0.0001 24 ± 11 T2*&lt; 10 ms (n. of pts) 0 - 7 T2* between 10.1 and 14 ms (n. of pts) 1 - 8 T2* between 14.1 and 20 ms (n. of pts) 7 - 8 T2* &gt; 20 ms (n. of pts) 25 - 35 T2*&lt; 10 ms (n. of pts) plus LVEF≤ 57 % 0/0 (0%) - 6/7 (85.7%) T2* between 10.1 and 14 ms (n. of pts) plus LVEF≤ 57 % 1/1 (100%) - 3/8 (37.5%) T2* between 14.1 and 20 ms (n. of pts) plus LVEF≤ 57 % 3/7 (42.8%) - 1/8 (12.5%) T2* &gt; 20 ms (n. of pts) plus LVEF≤ 57 % 7/25 (28%) - 5/35 (14.3%)

Onset of Cardiac Iron Loading in a Large and Homogeneous Cohort of Thalassemia Major Paediatric Patients

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2157-2157
Author(s):  
Alessia Pepe ◽  
Antonella Meloni ◽  
Brunella Favilli ◽  
Marcello Capra ◽  
Domenico Giuseppe D'Ascola ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Conflicts Of Interest ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Myocardial Iron ◽  
Cardiac Iron ◽  
Paediatric Patients ◽  
Biventricular Function ◽  
Significant Difference

Abstract Abstract 2157 Introduction: Magnetic Resonance Imaging (MRI) by the T2* technique allows highly reproducible and non invasive quantifications of myocardial iron burden and it is the gold standard for quantifying biventricular function parameters. It is important to determine the appropriate age to start MRI screening, because its high cost. Few data are available in the literature and they are contrasting. So the aim of this study was to address this issue in our paediatric patients with thalassemia major (TM). Methods: We studied retrospectively 72 patients (47 males, 4.2–17.9 years old, mean age 13.03 ± 3.70 years), enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network. Myocardial iron overload was measured by T2* multislice multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images. Results: The global heart T2* value was 29.7 ± 11.2 ms (range 6.2 – 48.0 ms). No significant correlation was found between global heart T2* value and age (see figure). The global heart T2* value did not show significant differences according to the sex (male 30.2 ± 11.0 ms versus female 28.7 ± 11.8 ms, P=0.568). Sixteen patients (22%) showed an abnormal global heart T2* value (<20 ms) and none of them was under 8 years of age. Global heart T2* value was negatively correlated with mean serum ferritin levels. Odds Ratio for high serum ferritin levels (≥ 1500 ng/ml) was 8.4 (1.01–69.37, OR 95%CI) for abnormal global heart T2* values (< 20 ms). The global heart T2* value did not show a significant difference with respect to the chelation therapy (P=0.322). No significant correlations were found between the global heart T2* values and the bi-atrial areas or the LV and RV morphological and functional parameters. Eight patients showed a left ventricular (LV) ejection fraction (EF) < 57% and none of them was under 7 years of age. Two patients showed a right ventricular (RV) EF < 52% and none of them was under 14 years of age. Conclusion: The MRI screening for both cardiac iron overload and function assessment can be started for TM patients at the age of 7 years. At this age not sedation is generally needed. If the availability of cardiac MRI is low, the serum ferritin levels could be used as a discriminating factor. Disclosures: No relevant conflicts of interest to declare.

Left Ventricular Volumes, Mass and Function Normalized to the Body Surface Area, Age and Gender From CMR in a Large Cohort of Well-Treated Thalassemia Major Patients without Myocardial Iron Overload

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 1090-1090
Author(s):  
Antonella Meloni ◽  
Giovanni Aquaro ◽  
Pierluigi Festa ◽  
Francesco Gagliardotto ◽  
Angelo Zuccarelli ◽  
...  
Keyword(s):  
Iron Overload ◽  
Body Surface ◽  
Healthy Subjects ◽  
Age Groups ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Large Cohort ◽  
Myocardial Iron ◽  
Lv Volumes ◽  
Age And Sex

Abstract Abstract 1090 Introduction. Cardiovascular Magnetic Resonance (CMR) allows an accurate and reproducible quantification of left ventricular (LV) parameters. In Thalassemia major (TM) patients different “normal” LV values have been reported due to chronic anemia and eventually pre-existing iron burdens. Moreover, in this population it is unknown the influence of sex and age on LV parameters and no ranges of normal have been reported using MASS® software. The aim of this study was to establish the ranges for normal LV volumes, mass and ejection fraction normalized to the influence of body surface area (BSA), age and sex from CMR in a large cohort of well-treated TM patients without myocardial iron overload. Methods. Among the 923 TM patients who underwent CMR within the MIOT network for the assessment of cardiac iron overload, function and fibrosis, we selected 142 patients with no known risk factors or history of cardiac disease, normal electrocardiogram, no myocardial fibrosis and no myocardial iron overload (all the cardiac segments with a normal T2* value). All patients had been regularly transfused and chelated since early childhood. Moreover, we studied 71 healthy subjects matched for age and sex. LV function parameters were quantitatively evaluated in a standard way by SSFP cine images using MASS® software. LV end-diastolic volume, end-systolic volume, stroke volume, and mass were normalized to BSA (EDVI, ESVI, SVI, massI). Results. The table shows the comparison of the CMR parameters with differentiation for sex and age in TM patients and healthy subjects and the cut-off of normality defined as mean – or + 2 standard deviation (SD). TM patients showed significantly lower BSA than the controls (P<0.0001). Significantly higher EDVI and SVI were found only for males < 14 years and > 30 years. Significantly higher LVEF were found only for males < 14 years. In TM patients all LV volumes indexes were significantly larger in males than in females (P<0.0001 in all cases). The EF was not different between the sexes. In males the ESVI and the EF were significant different among the age groups (P=0.006 and P=0.001, respectively). In females no significant differences were detected among the age groups. <14 14–20 20–30 30–40 >=40 TM H P TM H P TM H P TM H P TM H P Males N=7 N=7 N=6 N=6 N=25 N=15 N=23 N=11 N=6 N=5 EDVI (ml/m2) 94 ± 18 (130) 75 ± 11 0.034 96 ± 20 (136) 91 ± 20 0.715 103 ± 17 (137) 101 ± 13 0.686 92 ± 15 (122) 80 ± 11 0.022 94 ± 9 (112) 75 ± 11 0.013 ESVI (ml/m2) 31 ± 6 (43) 24 ± 6 0.058 38 ± 8 (54) 35 ± 14 0.670 38 ± 8 (54) 39 ± 9 0.676 32 ± 6 (44) 29 ± 6 0.160 29 ± 6 (41) 28 ± 10 0.816 SVI (ml/m2) 63 ± 14 (91) 51 ± 7 0.050 57 ± 12 (81) 56 ± 8 0.868 65 ± 10 (85) 62 ± 9 0.433 59 ± 10 (79) 51 ± 10 0.027 64 ± 8 (80) 47 ± 8 0.007 Mass I (g/m2) 57 ± 7 (71) 68 ± 5 0.007 57 ± 13 (83) 71 ± 7 0.043 66 ± 12 (90) 77 ± 12 0.006 62 ± 12 (86) 66 ± 10 0.184 68 ± 16 (100) 74 ± 11 0.536 EF (%) 66 ± 4 (58) 54 ± 6 <0.0001 60 ± 2 (56) 66 ± 14 0.322 63 ± 3 (57) 62 ± 6 0.520 65 ± 3 (59) 65 ± 8 0.972 68 ± 6 (56) 62 ± 9 0.234 Females N=2 N=2 N=8 N=6 N=24 N=6 N=33 N=8 N=8 N=5 EDVI (ml/m2) 63 ± 8 (79) 62 ± 4 0.951 81 ± 8 (97) 80 ± 9 0.866 83 ± 16 (115) 78 ± 9 0.499 77 ± 11 (99) 79 ± 10 0.669 82 ± 19 (120) 77 ± 16 0.614 ESVI (ml/m2) 23 ± 1 (25) 22 ± 8 0.823 30 ± 6 (42) 31 ± 3 0.789 30 ± 8 (46) 30 ± 4 0.883 26 ± 6 (38) 29 ± 7 0.216 28 ± 8 (44) 28 ± 12 0.974 SVI (ml/m2) 40 ± 7 (54) 41 ± 4 0.919 49 ± 3 (55) 49 ± 6 0.892 53 ± 9 (71) 48 ± 6 0.234 51 ± 6 (63) 50 ± 8 0.915 54 ± 11 (76) 49 ± 6 0.394 Mass I (g/m2) 34 ± 3 (40) 59 ± 19 0.296 47 ± 8 (63) 56 ± 6 0.040 53 ± 9 (71) 54 ± 12 0.893 52 ± 9 (70) 55 ± 13 0.465 51 ± 12 (75) 56 ± 5 0.344 EF (%) 63 ± 3 (57) 46 ± 17 0.388 63 ± 4 (55) 62 ± 4 0.694 65 ± 5 (55) 62 ± 3 0.137 66 ± 5 (56) 63 ± 7 0.271 66 ± 4 (58) 63 ± 7 0.254 Conclusions. In a large cohort of well-treated TM patients significant differences in LV parameters compared to controls were limited to males < 14 years and > 30 years. Due to the influence of BSA, sex and age, appropriate “normal” reference ranges normalized these variables should be used to avoid misdiagnosis of cardiomiopathy in TM patients. Disclosures: No relevant conflicts of interest to declare.

Regional Myocardial Contractility In Thalassemia Major By Magnetic Resonance Tagging

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4708-4708
Author(s):  
Antonella Meloni ◽  
Chiara Tudisca ◽  
Emanuele Grassedonio ◽  
Giancarlo Izzi ◽  
Maddalena Lendini ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Iron Overload ◽  
Myocardial Contractility ◽  
Healthy Subjects ◽  
Conflicts Of Interest ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Myocardial Iron ◽  
Cardiac Iron ◽  
Biventricular Function

Introduction Magnetic resonance (MR) tagging analyzed by dedicated tracking algorithms allows very precise measurements of myocardial motion and characterization of regional myocardial function. No extensive data are available in literature. Our aim was to quantitatively assess for the regional myocardial contractility in thalassemia major (TM) patients and to correlate it with heart iron overload and global biventricular function. Methods Seventy-four TM patients (46 F; 31.8 ± 8.5 yrs) enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network underwent MR (1.5T). Three short-axis (basal, medial and apical) tagged MR images were analyzed off-line using harmonic phase (HARP) methods (Diagnosoft software) and the circumferential shortening (Ecc) was evaluated for all the 16 myocardial segments. Four main circumferential regions (anterior, septal, inferior, and lateral) were defined. The same axes were acquired by a T2* GRE multiecho technique to assess myocardial iron overload (MIO). Biventricular function parameters were quantitatively evaluated by cine images. Results Segmental ECC values ranged from -9.66 ± 4.17 % (basal anteroseptal segment) to 13.36 ± 4.57 % (mid-anterior segment). No significant circumferential variability was detected. Compared with previous studied healthy subjects, TM patients showed strain values significantly lower in all the circumferential regions at each level (mean difference from 4 % to 13 %; P<0.001 for all the comparisons). Segmental Ecc values were not significantly correlated with the correspondent T2* values and no correlation was detected considering the global values, averaged over all segmental values. Three groups identified on the basis of cardiac iron distribution: no MIO, heterogenous MIO and homogeneous MIO. The global ECC was comparable among the three groups (-11.56 ± 1.60 % vs -11.70 ± 2.43 % vs -11.14 ± 1.95 %; P=0.602). Global ECC values were not significantly correlated with age and were comparable between the sexes. Circumferential shortening was not associated to left ventricular (LV) volumes and ejection fraction (with a P>0.5 in all the comparisons). Conclusions TM patients showed a significantly lower cardiac contractility compared with healthy subjects, but this altered contractility was not related to cardiac iron, volumes and function. Disclosures: No relevant conflicts of interest to declare.

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