Prognostic value of multiparametric cardiac magnetic resonance in sickle cell patients

Meloni, Antonella; Pistoia, Laura; Quota, Alessandra; Messina, Giuseppe; Ricchi, Paolo; Bagnato, Sergio; Gerardi, Calogera; Lisi, Roberto; Cuccia, Liana; Renne, Stefania; Vallone, Antonino; Righi, Riccardo; Positano, Vincenzo; Pepe, Alessia; Cademartiri, Filippo

doi:10.1007/s00277-022-05057-6

Cited by 4 publications

(6 citation statements)

References 69 publications

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“…Moreover, the RV’s morphology has been demonstrated to be associated with known processes implicated in the pathogenesis of atrial fibrillation, like systemic inflammation and neurohormonal activation, which influence myocardial fibrosis, atrial stretch, and modulation of ionic channel function [ 52 , 53 ]. Importantly, an increased RV mass index also demonstrated a significant prognostic value in patients affected by sickle-cell disease [ 54 ]. Although our results need to be validated and confirmed in further studies, they warrant including the RV mass assessment into the routine CMR of patients with hemoglobinopathies.…”

Section: Discussionmentioning

confidence: 99%

Prognostic Role of Multiparametric Cardiac Magnetic Resonance in Neo Transfusion-Dependent Thalassemia

Meloni,

Pistoia,

Ricchi

et al. 2024

JCM

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Background: We prospectively evaluated the predictive value of multiparametric cardiac magnetic resonance (CMR) for cardiovascular complications in non-transfusion-dependent β-thalassemia (β-NTDT) patients who started regular transfusions in late childhood/adulthood (neo β-TDT). Methods: We considered 180 patients (38.25 ± 11.24 years; 106 females). CMR was used to quantify cardiac iron overload, biventricular function, and atrial dimensions, and to detect left ventricular (LV) replacement fibrosis. Results: During a mean follow-up of 76.87 ± 41.60 months, 18 (10.0%) cardiovascular events were recorded: 2 heart failures, 13 arrhythmias (10 supraventricular), and 3 cases of pulmonary hypertension. Right ventricular (RV) end-diastolic volume index (EDVI), RV mass index (MI), LV replacement fibrosis, and right atrial (RA) area index emerged as significant univariate prognosticators of cardiovascular complications. The low number of events prevented us from performing a multivariable analysis including all univariable predictors simultaneously. Firstly, a multivariable analysis including the two RV size parameters (mass and volume) was carried out, and only the RV MI was proven to independently predict cardiovascular diseases. Then, a multivariable analysis, including RV MI, RA atrial area, and LV replacement fibrosis, was conducted. In this model, RV MI and LV replacement fibrosis emerged as independent predictors of cardiovascular outcomes (RV MI: hazard ratio (HR) = 1.18; LV replacement fibrosis: HR = 6.26). Conclusions: Our results highlight the importance of CMR in cardiovascular risk stratification.

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Section: Discussionmentioning

confidence: 99%

Prognostic Role of Multiparametric Cardiac Magnetic Resonance in Neo Transfusion-Dependent Thalassemia

Meloni,

Pistoia,

Ricchi

et al. 2024

JCM

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“…The severity of symptoms and the treatment approach can vary, depending on the specific genetic mutations involved [8,9]. There is a lack of studies specifically documenting the phenotype of Sβ-thal patients; in fact, Sβ-thal patients have often been included in studies on homozygous SCD patients, based on a similar clinical course [10].…”

Section: Introductionmentioning

confidence: 99%

“…The organ-specific involvement in SCD and beta thalassemia patients has been extensively described. Cardiovascular impairment represents an important cause of morbidity and mortality both in SCD and in beta thalassemia [10,11]. Both syndromes lead to a chronic hemolysis-related anemia and a compensatory rise in blood volume, which enhances the cardiac output state [12].…”

Section: Introductionmentioning

confidence: 99%

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Multiparametric Cardiac Magnetic Resonance Assessment in Sickle Beta Thalassemia

Pistoia,

Meloni,

Positano

et al. 2024

Diagnostics

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Cardiac involvement in sickle beta thalassemia (Sβ-thal) patients has been poorly investigated. We aimed to evaluate cardiac function and myocardial iron overload by cardiovascular magnetic resonance (CMR) in patients with Sβ-thal. One-hundred and eleven Sβ-thal patients consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network were studied and compared with 46 sickle cell anemia (SCA) patients and with 111 gender- and age- matched healthy volunteers. Cine images were acquired to quantify biventricular function. Myocardial iron overload (MIO) was assessed by the T2* technique, while macroscopic myocardial fibrosis was evaluated by the late gadolinium enhancement (LGE) technique. In Sβ-thal and SCA patients, the morphological and functional CMR parameters were not significantly different, except for the left atrial area and left ventricular (LV) stroke volume, indexed by body surface area (p = 0.023 and p = 0.048, respectively), which were significantly higher in SCA patients. No significant differences between the two groups were found in terms of myocardial iron overload and macroscopic myocardial fibrosis. When compared to healthy subjects, Sβ-thal patients showed significantly higher bi-atrial and biventricular parameters, except for LV ejection fraction, which was significantly lower. The CMR analysis confirmed that Sβ-thal and SCA patients are phenotypically similar. Since Sβ-thal patients showed markedly different morphological and functional indices from healthy subjects, it would be useful to identify Sβ-thal/SCA-specific bi-atrial and biventricular reference values.

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“…When describing red blood cells with IDA, tiny erythrocytes are frequently used (i.e., corpuscular volume smaller than 80 m3). 6,7 Hypopigmented, although the signs of iron deficiency show up gradually. Patients who have serum ferritin levels below 25 mcg per mL (25 mcg per L) and serum iron levels below 60 mcg/dL are very likely to be anemic.…”

Section: Introductionmentioning

confidence: 99%

Clinical Significance of Serum Iron in the Diagnosis of Iron Deficiency Anemia in Patients from Local Population of Punjab-Lahore

Farheen¹,

Malik²,

Anwar³

et al. 2023

PJMHS

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Background: The main cause of anemia in one-third of the world's population is a lack of iron (ID). Anemia affects between 700 and 800 million individuals globally. Methodology: Only the patients diagnosed with iron deficiency anemia was selected from Ali Fatima Hospital Lahore and Jinnah Hospital Lahore for determination of serum iron concentration in comparison with healthy controls. Results: In our study male patients were 67% and females were 33%. Frequency of children with beta thalassemia major under 5 year of age is 19%, 5-9 year is 24%, 9-15 years is 31% and above 15 years is 26% Practical implications: The current study aims to spread the awareness among community about clinical significance of serum iron in the diagnosis of iron deficiency anemia in patients and ways to control the iron deficiency. This investigation will assist in determining the severity of the condition in our community as well as the status of serum iron levels in individuals with iron deficiency anemia. The evaluation of serum iron levels in anemic individuals will be helpful to medical experts in determining the best course of treatment. Conclusion: We concluded that renal tubular damage and hepatocellular injury may be secondary to oxidative lipid peroxidation mediated by the iron overload. However further investigations are extremely necessary for this interpretation. A better evaluation of iron toxicity and regular monitoring of renal functions, liver functions, accompanied by an improvement in chelation therapy along with the administration of selective antioxidants (vitamins E and C), may protect different organs against oxidative stress and its consequences, specifically endocrine problems. Keywords: Anemia, Iron deficiency, Ferritin, Hemoglobin, Thalassemia, hepatocellular injury.

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Prognostic value of multiparametric cardiac magnetic resonance in sickle cell patients

Cited by 4 publications

References 69 publications

Prognostic Role of Multiparametric Cardiac Magnetic Resonance in Neo Transfusion-Dependent Thalassemia

Prognostic Role of Multiparametric Cardiac Magnetic Resonance in Neo Transfusion-Dependent Thalassemia

Multiparametric Cardiac Magnetic Resonance Assessment in Sickle Beta Thalassemia

Clinical Significance of Serum Iron in the Diagnosis of Iron Deficiency Anemia in Patients from Local Population of Punjab-Lahore

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