Β-thalassemia Major Patients Research Articles

The Role of Erythropoiesis on Hepcidin Level in Polytransfused b-Thalassemia Major Patients

AbstractBackground: Hepcidin peptide hormone is a main con-troller of iron homeostasis, itbecomes elevated in case of iron overload, however despite iron overload in b-Thalassemia major (b-TM) patients a contradictory decrease in Hepcidin occurs. The incompatible Hepcidin level is the main responsible factor causing the iron overload status in iron-loading anemias such as (b-TM) which contributes to organ dysfunctionand to iron toxicity.Aim of Study: To evaluate the conflicting effect of increased erythropoiesis in contrast to the effect of iron overloading on Hepcidin serum level and its correlation with iron status in (b-TM) patients.Subjects and Methods: For all patients and controls Complete Blood Count (CBC), serum assaysof Hepcidin, iron, ferritin, transferrin, HCVAbs, HBs Ag, CRP and serum level were performed.Results: Hepcidin level was significantly lowered in (b-TM) patients compared to controls with high significant difference (p=0.00). There was no correlation between serum Hepcidin and ferritin level, neither was between Hepcidin and serum iron, transferrin, Hb level and reticulocyte count in the study group. Hepcidin/Ferritin ratio showed high significance difference (p<0.000) with mean value in the study group 0.056 vs. 4.75 in controls pointing to an incom-patible levels of Hepcidin to iron overload status. Hepcidin level wasnegative correlatedwith age (p<0.05).Conclusion: Hepcidin was markedly decreased in the study group with no significant correlation between serum Hepcidin and ferritin level as a marker of iron overload in thalassemia major. Hepcidin deficiency is the main contributing factor of iron overload in b thalassemia which results from a strong suppressive effect of the high erythropoietic activity on Hepcidin expression. Hepcidin-ferritin ratio was markedly depressed <1 in all b thalassemia major patients which indicating suppression of Hepcidin out of proportion with the degree of iron loading.

Effects of Splenectomy before Transplantation on β-Thalassaemia Major with Matched Sibling and Unrelated Hematopoietic Stem Cell Transplantation

Purpose:Splenectomy can improve the quality of life of patients with β-thalassaemia major to some extent, but also increase some unpredictable risk.In order to understand the effects of splenectomy before transplantation on hematopoietic stem cell transplantation reconstruction time, infection, graft versus host disease and hepatic veno-occlusive disease of matched sibling and unrelated hematopoietic stem cell transplantation, the following studies were conducted.Methods:A retrospective analysis of 80 β-thalassaemia major patients in the southern hospital between January 2012 and December 2017 for matched hematopoietic stem cell transplantation, and 35 of them had a splenectomy before the transplant. Cox model adjustment, t test and χ2 test were used to compare the incidence of neutrophil implantation time, hemoglobin implantation time, platelet implantation time, infection, graft versus host disease and hepatic vein occlusion.Results:Neutrophilic granulocytes and platelets were implanted more rapidly in patients who underwent splenectomy than those who did not (P < 0.05).There was no significant difference in the incidence of infection, graft versus host disease and hepatic vein occlusion.Conclusion: For level of Ⅱ-Ⅲ β-thalassaemia major patients, hypersplenism can cause delayed reconstruction of hematopoietic stem cell transplantation, while before transplantation splenectomy to speed up the implantation of hematopoietic stem cell transplantation, and does not increase the incidence of complications after transplantation. DisclosuresNo relevant conflicts of interest to declare.

A Randomized Prospective Study to Assess the Effect of Vitamin E on Peroxiredoxin- 2 As a Novel Antioxidant Marker in Young Beta-Thalassemia Major Patients on Different Chelation Therapy

Introduction: Oxidative stress plays a central role in the pathogenesis of morbidities in β-thalassemia. Peroxiredoxin-2 (PRDX2) is the third most abundant cytoplasmic protein in red cells and is able to reduce and detoxify a vast range of organic peroxides, H2O2, and peroxynitrite. PRDX2 has been demonstrated to be induced by oxidative stress and that cells overexpressing PRDX2 are more resistant to the oxidative stress. Vitamin E is a fat-soluble vitamin and an anti-oxidant which is often depleted in thalassemia patients as a result of iron overload. Aim: This randomized prospective trial aimed to investigate the efficacy and safety of vitamin E as an adjuvant therapy to the three used iron chelators in moderately iron overloaded young vitamin E-deficient β-thalassemia major (β-TM) patients in relation to tissue iron overload and examine its potential corrective value to markers of oxidative stress and to the level of PRDX2 as a novel protective marker of oxidative stress. Methods: Inclusion criteria were β-TM patients aged 6-18 years, vitamin E deficient, serum ferritin (SF) >1000-2500 μg/L and cardiac T2* > 10 ms and ejection fraction > 56 %. A total of 245 β-TM patients were screened for eligibility, 180 were enrolled while 35 did not meet inclusion criteria and 30 were excluded. The 180 β-TM vitamin E-deficient patients were equally enrolled into 3 groups (each; n=60) and received desferrioxamine (DFO), deferiprone (DFP) and deferasirox (DFX). Patients in each group were further randomized either to receive vitamin E supplementation (400 IU daily) or not (n=30). All patients received vitamin E (group A) or no vitamin E (group B) were followed-up for one year with assessment of transfusion index, hemoglobin, serum ferritin (SF), liver iron content (LIC) and cardiac magnetic resonance imaging (MRI) T2*. Malondialdehyde (MDA), as an index for lipid peroxidation, and antioxidants including reduced glutathione (GSH), superoxide dismutase (SOD), glutathione peroxidase (GPX), catalase and PRXD2 were assessed. Results: Baseline vitamin E was negatively correlated to SF, LIC and MDA while it was positively correlated to GSH, GPX, catalase and PRXD2. No significant difference was found as regards the studied variables at baseline and study end in patients who did not receive vitamin E (group B). After vitamin E therapy, transfusion index, SF, and LIC were significantly decreased in group A patients while hemoglobin and cardiac MRI T2* were elevated compared with baseline levels or those in group B without vitamin E. MDA levels were decreased while all the studied antioxidants were increased post-vitamin E supplementation compared with baseline levels or those in group B without vitamin E. The same improvement was found among DFP-treated patients post-vitamin E compared with baseline data. DFP-treated patients had the highest hemoglobin and antioxidant levels with the lowest SF and LIC compared with DFO or DFX subgroups. Conclusions: Vitamin E as an adjuvant therapy possibly potentiates the efficacy of DFP more than DFO and DFX in reducing iron burden and reduces oxidative stress in the moderately iron overloaded vitamin E-deficient β-TM patients, with no adverse events. DisclosuresNo relevant conflicts of interest to declare.

Quality of Life and Depression in Turkish Patients with β-Thalassemia Major: A Cross-Sectional Study

Thalassemias are the most common monogenic disorders worldwide. Thalassemia patients experience difficulties in their schooling, finding jobs and/or marriage because of functional and physical limitations caused by this disease. It is expected that the quality of life (QoL) of patients with thalassemia will be lower than those without this disease. The aim of this study was to benefit worldwide thalassemia patients in terms of QoL and mental health. This cross-sectional study was performed in Turkey. The study population consisted of of 57 β-thalassemia major (β-TM) patients and the control group. The short form-36 (SF-36) questionnaire and Beck depression inventory (BDI) were used. The mean age of the patients was 21.6 ± 6.6 (age range 15-39) and the male-to-female ratio was 0.7. The mean SF-36 scores of the patient and the control groups were 59.2 ± 12.4 and 75.7 ± 11.8, and the mean BDI scores of the patients and controls were 13.5 ± 6.4 and 6.1 ± 3.7, respectively. There was a statistically significant difference between the total SF-36 and BDI scores of patients and controls. We aimed to investigate the effects of the decrease in morbidity and mortality of β-thalassemia (β-thal) due to regular transfusions and chelation therapy on the QoL and mental health of patients. The β-TM patients have a comparatively worse QoL score than the normal population. Improving QoL should be the target of clinicians who are monitoring adolescent or young adult β-TM patients.

The relationship between ferritin levels and oxidative stress parameters in serum of β-thalassemia major patients

The relationship between ferritin levels and oxidative stress parameters in serum of β-thalassemia major patients

Role of CYP1A1, ABCG2, CYP24A1 and VDR gene polymorphisms on the evaluation of cardiac iron overload in thalassaemia patients.

Iron-burden-induced arrhythmia and heart failure are among the leading causes of morbidity and mortality in β-thalassaemia major patients. T2* cardiac magnetic resonance remains the only reliable noninvasive method for the heart iron excess assessment. We explored the role of single nucleotide polymorphisms involved in vitamin D metabolism, transport and activity and in deferasirox (DFX) metabolism on cardiac iron burden. One hundred and five β-thalassaemia patients, treated with DFX, were enrolled in the present study. Drug plasma Ctrough was measured by a high-performance liquid chromatography-ultraviolet method. Allelic discrimination was carried out using the real-time PCR. CYP1A1*1189 CC, ABCG2 421 GA, CYP24A1 8620 GG and VDR TaqI CC single nucleotide polymorphisms influenced T2* values. Age, serum ferritin, ABCG2 421 GA, ABCG2 1194 +928 TC/CC, CYP24A1 22776 TT and VDR TaqI TC/CC were retained in linear regression model. Our results suggested, for the first time, the role of DFX and vitamin D pharmacogenetics on cardiac iron overload.

Effect of serum fibroblast growth factor receptor 2 and CAPS proteins on calcium status in β-thalassaemia major patients who are free from overt inflammation

Bone disorders and disturbed calcium (Ca) homeostasis are common disorders in β-thalassaemia major (β-TM). In the present study, two bone related markers are studied in β-TM patients with negative C-reactive protein for the first time; fibroblast growth factor receptor 2 (FGFR2) and CAPS protein. Another goal is to estimate the correlation between the recent parameters and bone biomaterials as a function of iron status parameters in β-TM patients. The results revealed that, in patients with β-TM serum FGFR2, CAPS, alkaline phosphatase (ALP) and Mg significantly increased while serum Ca levels were low as compared with controls. Ca status is correlated with iron overload in β-TM. A significant correlation was present between CAPS and FGFR2. In conclusion, FGFR2 and CAPS associated with Ca status and subsequent bone disturbances in β-TM patients. Their level can be predicted from the equation: CAPS =0.001ALP +0.48FGFR2-1.26Ca – 3.95Pi +12.76 with acceptable applicability.

Biochemical Status of Beta-Thalassemia Major Patients in Erbil City

Background and objectives: β -thalassemia major patient is one of the hereditary hemolytic diseases, which can cause many hematological and biochemical changes in the affected patient. And these changes can happen even when the patient is treated adequately. The objective was to study biochemical changes in the level of serum hepcidin, osteocalcin, calcium, ferritin, iron, PTH and IL-6 in patients with β- thalassemia major and to compare it with control subjects. Patients and method: In this research 40 patients with beta thalassemia major, 20 Female and 20 Male (age ranged from 10 to 38 years), and 40 control subjects 20 Female and 20 Male (age ranged from 9 to 33 years) were studied. Measurement of serum hepcidin, osteocalcin, calcium, ferritin, iron, PTH and IL-6 were done by the researcher for both cases and control groups. Results: Serum Ferritin and Iron were higher significantly in all thalassemic patients (P< 0.001), this increment was proportional with increasing number of units of blood transfusion and aging. Mean serum Hepcidin, PTH, Osteocalcin and IL6 were significantly lower in thalassemic patients in contrast to the control subjects (P< 0.001). Reduction in S-PTH was proportional to increasing number of blood transfusion and aging. Conclusion: Our study demonstrates that in B-thalassemia major patients Serum Ferritin and Iron were increased proportionally with increasing age and number of units of blood transfusion. Mean serum PTH, osteocalcin, hepcidin, and IL-6 were reduced, but mean serum calcium was remained normal.

An investigation of the effects of curcumin on iron overload, hepcidin level, and liver function in β-thalassemia major patients: A double-blind randomized controlled clinical trial.

This study investigated the effects of curcumin, the active polyphenol in turmeric, on iron overload, hepcidin level, and liver function in β-thalassemia major patients. This double-blind randomized controlled clinical trial was conducted on 68 β-thalassemia major patients. The subjects were randomly divided into 2 groups to receive either 500mg curcumin capsules (total: 1,000mg) twice daily or placebo for 12weeks. Dietary intakes and biochemical variables including hemoglobin, transferrin saturation, total iron binding capacity, nontransferrin bound iron (NTBI), ferritin, hepcidin, alanine aminotransferase (ALT), and aspartate aminotransferase (AST) were assessed at the beginning and end of the trial. Curcumin significantly reduced serum levels of NTBI (2.83 ± 1.08 compared with 2.22 ± 0.97 μmol/L, p = .001), ALT (42.86 ± 11.15 compared with 40.60 ± 9.89 U/L, p = .018), and AST (49.45 ± 12.39 compared with 46.30 ± 10.85 U/L, p = .002) at the end of the study. Based on analysis of covariance, a significant decrease was also observed in levels of NTBI (2.22 ± 0.97 vs. 2.55 ± 0.94 μmol/L, p = .026), ALT (40.60 ± 9.89 vs. 45.01 ± 10.42 U/L, p = .004), and AST (46.30 ± 10.85 vs. 50.99 ± 9.36 U/L, p = .009) in curcumin group in comparison with placebo group. There were no significant changes in hepcidin and other variables in any of the 2 groups. Curcumin administration alleviated iron burden and liver dysfunction by reducing NTBI, ALT, and AST levels in patients with β-thalassemia major.

High ferritin in homozygous and heterozygous β-thalassemia tends to decrease oxidative stress levels

BackgroundIneffective erythropoiesis and multiple blood transfusions may cause iron overload, leading to high level of ferritin in β-thalassemia patients. Iron has the ability to catalyze the production of reactive oxygen species (ROS), which can be prevented by an adequate activity of superoxide dismutase (SOD) and glutathione peroxidase (GPx). The aim of this study was to explore the association between high ferritin levels and oxidative stress among β-thalassemia patients by measuring SOD and GPx levels. MethodsThis was a cross sectional study among β-thalassemia major patients. Ferritin, SOD, and GPx were measured and compared between β-thalassemia patients with homozygous IVS1nt5 and heterozygous IVS1nt5/HbE mutations, which were the most prevalent mutations found in our previous study. The Spearman correlation test was used to analyze the data. ResultsThe ferritin levels in all β-thalassemia major patients were very high (range: 791-12,340 µg/L). However, no significant differences were observed (p=0.318) between homozygous IVS1nt5 (n=45) compared to heterozygous IVS1nt5/HbE (n=13). The oxidative stress markers SOD and GPx were not significantly different between homozygous IVS1nt5 and heterozygous IVS1nt5/HbE (p=0.450 and p=0.323, respectively). The correlations between ferritin and SOD and GPx oxidative stress levels were not significant in both homozygous IVS1nt5 and heterozygous IVS1nt5/HbE mutations. ConclusionHigh ferritin levels in β-thalassemia patients tend to decrease the GPx level in all thalassemia patients and SOD level in half of the patients, indicating that GPx and SOD may play a role in the occurrence of oxidative stress among thalassemia patients. The mechanism of oxidative stress in thalassemia needs to be further explored.

Atrial fibrillation in β-thalassemia Major Patients: Diagnosis, Management and Therapeutic Options

The prevalence of atrial fibrillation (AFib) in β-thalassemia major (β-TM) patients has increased in the last few years, reaching up to 33.0%. Several factors may drive this value to even more in the next few years. We summarized the main challenges in the management and therapy of AFib in this very specific group of patients.

Diabetes Mellitus and Impaired Glucose Tolerance in β-Thalassaemia Major Patients with Secondary Haemchromatosis: Frequency and their Associations from a Developing Country

Diabetes Mellitus and Impaired Glucose Tolerance in β-Thalassaemia Major Patients with Secondary Haemchromatosis: Frequency and their Associations from a Developing Country

Renal iron deposition by magnetic resonance imaging in pediatric β-thalassemia major patients: Relation to renal biomarkers, total body iron and chelation therapy

BackgroundThe reciprocal of multiecho gradient-echo (ME–GRE) T2* magnetic resonance imaging (MRI) R2*, rises linearly with tissue iron concentration in both heart and liver. Little is known about renal iron deposition in β-thalassemia major (β-TM). AimTo assess renal iron overload by MRI and its relation to total body iron and renal function among 50 pediatric patients with β-TM. MethodsSerum ferritin, serum cystatin C, urinary albumin creatinine ratio (UACR), and urinary β2-microglobulin (β2 M) were measured with calculation of β2 M/albumin ratio. Quantification of liver, heart and kidney iron overload was done by MRI. ResultsSerum cystatin C, UACR and urinary β2 microglobulin as well as urinary β2m/albumin were significantly higher in β-TM patients than the control group. No significant difference was found as regards renal R2* between Patients with mean serum ferritin above 2500 μg/L and those with lower serum cutoff. Renal R2* was higher in patients with poor compliance to chelation therapy and positively correlated to indirect bilirubin, LDH, cystatin C and LIC but inversely correlated to cardiac T2*. Conclusionkidney iron deposition impairs renal glomerular and tubular functions in pediatric patients with β-TM and is related to hemolysis, total body iron overload and poor compliance to chelation.

Relationship between uric acid levels and cardiometabolic findings in a large cohort of β-thalassemia major patients.

to evaluate the relationship between uric acid (UA), hepatic and cardiac iron overload (T2*-MRI), ferritin, endocrinological diseases and cardiac complications in a large thalassemia major (TM) cohort. A total of369 TM patients (187 men; 33±6years) were retrospectively studied, from the myocardial iron overload in thalassemia (MIOT) electronic databank. Multiple regression model identified male sex (p<0.001), BMI (p<0.001) and T2* (p≤0.001) as UA independent correlates. Moreover, UA and derivatives of reactive oxygen species (an oxidative index; r=-0.3; p≤0.05) are inversely correlated. Conversely, the multivariate logistic analysis identified low UA (NANHES-III criteria) as one independent predictor for low global heart T2* (p<0.5) together with liver iron concentrations (>3mg/g/dw), heart failure, endocrinopathies, ferritin (>2000ng/l), alanine transaminase (>40 UI/l) and/or aspartate transaminase (>35 UI/l) and/or glutamyl transferase (>64 UI/l). UA appears directly associated to T2* and inversely with derivatives of reactive oxygen species, and as such reduced according to increased oxidative stress and cardiac iron overload in TM patients.

Modified conditioning regimen improves outcomes of unrelated donor peripheral blood stem cell transplantation for β-thalassaemia major patients.

The objective of this study was to evaluate the feasibility of a modified conditioning regimen for the treatment of patients with β-thalassaemia major (TM), using unrelated donor peripheral blood stem cell transplantation (UD-PBSCT). A modified conditioning regimen based on intravenous busulfan, cyclophosphamide, fludarabine, and antithymocyte globulin was performed in 50 consecutive childhood patients with β-TM and a median age of 4.6 years (range, 2-12 years). According to Pesaro's classification, three classes of risk are identified using the criteria of degree of hepatomegaly, portal fibrosis, and quality of the chelation treatment. Patients with three adverse criteria constituted class III, none of the adverse criteria constituted class I, and one or two of the adverse criteria formed class II. Ten patients were class I, 36 class II, and four class III. All patients were transplanted with UDs containing 37 of 10/10 human leukocyte antigen (HLA)-matched pairs, 11 of 9/10 matched pairs, and two of 8/10 matched pairs. The median follow-up was 36 months (range, 9-96 months). All patients successfully achieved engraftment, two of whom developed persistentthrombocytopaenia. The incidence of acute graft-versus-host disease (aGVHD) grade III-IV and chronic graft-versus-host disease (cGVHD) were 12% and 8%, respectively. However, 8.3% of HLA-matched and 15.4% of HLA-mismatched patients developed aGVHD. The incidence of severe bacterial infections and fungal pneumonia was 12% and 20%, respectively. The 3-year overall survival, disease-free survival, graft rejection, and transplant-related mortality were 94%, 92%, 2%, and 6%, respectively. This modified conditioning protocol effectively improved outcomes of UD-PBSCT for patients with β-TM.

Study of zinc and copper in patients with Beta Thalassemia major and splenctomized in Kirkuk city

Beta thalassemia major is one of the common diseases in the world; it is one of health problems among children in Iraq. The disease leads to hemolytic anemia due to reduction of beta globin chains. Zinc and copper are important trace elements for synthesis of metalloproteins of hemoglobin enzymes. As zinc and copper are essential trace for synthesis of DNA; it is important to show their effect on severity of the disease among thalassemic patients. This study was performed during the period from November 2014 to June 2015. A total of 100 β-thalassemia major patients were enrolled in the current study; and patients were from the Thalassemia center in Azadi Teaching Hospital and 30 healthy children were taken as a control group. The blood samples were collected from 46 males and 54 females of different ages, ranging from (1-18) years. The level of serum zinc and copper were measured using spectrophotometer method. Comparison between thalassemic patients with control group; the results shown that there was high significant increased (p>0.01) in serum level of copper (148.69±28.90) while in serum level of zinc was decreased in thalassemic patients (60.52±10.63). Statistically there was no significant difference between different age groups and gender. The copper and zinc values decreased in splenctomized patients in comparison with non-splenctomized patients. The present study concluded that the level of serum copper increased while the level of serum zinc decreased in β-thalassemia major patients, this is also confirmed by other studies.

Hemodynamic Functionality of Transfused Red Blood Cells in the Microcirculation of Blood Recipients.

The primary goal of red blood cell (RBC) transfusion is to supply oxygen to tissues and organs. However, due to a growing number of studies that have reported negative transfusion outcomes, including reduced blood perfusion, there is rising concern about the risks in blood transfusion. RBC are characterized by unique flow-affecting properties, specifically adherence to blood vessel wall endothelium, cell deformability, and self-aggregability, which define their hemodynamic functionality (HF), namely their potential to affect blood circulation. The role of the HF of RBC in blood circulation, particularly the microcirculation, has been documented in numerous studies with animal models. These studies indicate that the HF of transfused RBC (TRBC) plays an important role in the transfusion outcome. However, studies with animal models must be interpreted with reservations, as animal physiology may not reflect human physiology. To test this concept in humans, we have directly examined the effect of the HF of TRBC, as expressed by their deformability and adherence to vascular endothelium, on the transfusion-induced effect on the skin blood flow and hemoglobin increment in β-thalassemia major patients. The results demonstrated, for the first time in humans, that the TRBC HF is a potent effector of the transfusion outcome, expressed by the transfusion-induced increase in the recipients' hemoglobin level, and the change in the skin blood flow, indicating a link between the microcirculation and the survival of TRBC in the recipients' vascular system. The implication of these findings for blood transfusion practice and to vascular function in blood recipients is discussed.

Increased levels of advanced glycation end products positively correlate with iron overload and oxidative stress markers in patients with β-thalassemia major.

The impaired biosynthesis of the β-globin chain in β-thalassemia leads to the accumulation of unpaired alpha globin chains, failure in hemoglobin formation, and iron overload due to frequent blood transfusion. Iron excess causes oxidative stress and massive tissue injuries. Advanced glycation end products (AGEs) are harmful agents, and their production accelerates in oxidative conditions. This study was conducted on 45 patients with major β-thalassemia who received frequent blood transfusions and chelation therapy and were compared to 40 healthy subjects. Metabolic parameters including glycemic and iron indices, hepatic and renal functions tests, oxidative stress markers, and AGEs (carboxymethyl-lysine and pentosidine) levels were measured. All parameters were significantly increased in β-thalassemia compared to the control except for glutathione levels. Blood glucose, iron, serum ferritin, non-transferrin-bound iron (NTBI), MDA, soluble form of low-density lipoprotein receptor, glutathione peroxidase, total reactive oxygen species (ROS), and AGE levels were significantly higher in the β-thalassemia patients. Iron and ferritin showed a significant positive correlation with pentosidine (P < 0.01) but not with carboxymethyl-lysine. The NTBI was markedly increased in the β-thalassemia patients, and its levels correlated significantly with both carboxymethyl-lysine and pentosidine (P < 0.05). Our findings confirm the oxidative status generated by the iron overload in β-thalassemia major patients and highlight the enhanced formation of AGEs, which may play an important role in the pathogenesis of β-thalassemia major.

Pain in β-thalassemia major patients: an important yet neglected issue.

Pain in β-thalassemia major patients: an important yet neglected issue.

Left Ventricular Diastolic Dysfunction in β-Thalassemia Major with Heart Failure

We studied the clinical, electrocardiographic, echocardiographic, Doppler and T2* cardiac magnetic resonance (CMR) data of all adult β-thalassemia major (β-TM) patients with heart failure (HF) consecutively observed at our referral center of the Sicilian region between 2008 and 2016. There were 16 patients enrolled in the study. Echocardiographic examination showed that only one patient had HF with systolic dysfunction of the left ventricle (HFrEF), whereas the others had HF with preserved systolic function of the left ventricle (HFpEF). Systolic dysfunction of the right ventricle (RV) was observed in 13 cases. Furthermore, 30.0% of the patients presented T2* CMR values consistent with intermediate risk of systolic dysfunction of the left ventricle (LV) due to iron overload, whereas 70.0% had normal values. Typical electrocardiographic abnormalities (wide T wave inversion and low voltages) were observed in 11 out of 16 patients. In conclusion, in the adult β-TM patients with HF recently observed at our center, the predominant form was that with diastolic dysfunction of the LV, and with systolic dysfunction of the RV. Only 30.0% had low values of T2* CMR. Typical electrocardiographic abnormalities were found in 69.0%.