Right ventricular function in β-thalassemia children: comparing three-dimensional echocardiography with other functional parameters

El-Hawary, Eslam El-Sayed; Tolba, Osama; Elkaffas, Asmaa A; Shabana, Ahmed Hamdy

doi:10.1038/s41390-021-01900-0

Cited by 5 publications

(3 citation statements)

References 31 publications

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“…RV dysfunction is frequently described, although less compared to LV dysfunction, e.g., in long-term adult survivors of childhood lymphoma and acute lymphoblastic leukemia [27], hypertensive heart failure [28], and also in β-thalassemia children [29], but prior to our present study not in PA patients. RV systolic dysfunction is recognized as independent predictor of clinical outcome e.g., in ischemic heart disease or DCM [10][11][12][13].…”

Section: Discussionmentioning

confidence: 57%

Evaluation of Right Ventricular Function in Patients with Propionic Acidemia—A Cross-Sectional Study

et al. 2023

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(1) Background: In propionic acidemia (PA), myocardial involvement often leads to progressive cardiac dysfunction of the left ventricle (LV). Cardiomyopathy (CM) is an important contributor to mortality. Although known to be of prognostic value in CM, there are no published data on right ventricular (RV) function in PA patients. (2) Methods: In this cross-sectional single-center study, systolic and diastolic RV function of PA patients was assessed by echocardiography, including frequency, onset, and combinations of echocardiographic parameters, as well as correlations to LV size and function. (3) Results: N = 18 patients were enrolled. Tricuspid annulus S’ was abnormal in 16.7%, RV-longitudinal strain in 11.1%, tricuspid annular plane systolic excursion (TAPSE) in 11.1%, Tricuspid valve (TV) E/e’ in 33.3%, and TV E/A in 16.7%. The most prevalent combinations of pathological parameters were TV E/A + TV E/e’ and TAPSE + TV S’. With age, the probability of developing abnormal RV function increases according to age-dependent normative data. There is a significant correlation between TAPSE and mitral annular plane systolic excursion (MAPSE), and RV/LV-longitudinal strain (p ≤ 0.05). N = 5 individuals died 1.94 years (mean) after cardiac evaluation for this study, and all had abnormal RV functional parameters. (4) Conclusions: Signs of diastolic RV dysfunction can be found in up to one third of individuals, and systolic RV dysfunction in 16.7% of individuals in our cohort. RV function is impaired in PA patients with a poor outcome. RV functional parameters should be used to complement clinical and left ventricular echocardiographic findings.

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Section: Discussionmentioning

confidence: 57%

Evaluation of Right Ventricular Function in Patients with Propionic Acidemia—A Cross-Sectional Study

et al. 2023

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“…The ferritin test is a non-specific test providing different sensitivities and specificities at dissimilar cutoff points for evaluating cardiac and liver hemosiderosis in multiple studies. Clinical sequelae, such as heart failure, cardiac dysrhythmia, and pulmonary hypertension from transfusional iron overload, still prevail in β-thalassemia patients 6 , 13 , 14 . In contrast, no consensus exists on the optimum cutoff of ferritin to screen for cardiac and liver hemosiderosis.…”

Section: Introductionmentioning

confidence: 99%

Ferritin thresholds for cardiac and liver hemosiderosis in β-thalassemia patients: a diagnostic accuracy study

Darvishi‐Khezri

Aliasgharian

Naderisorki

et al. 2022

Sci Rep

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Ferritin is frequently used to screen some dire consequences of iron overload in β-thalassemia patients. The study aimed to define the best cutoff point of ferritin to screen for cardiac and liver hemosiderosis in these cases. This was a registry-based study on β-thalassemia patients living throughout Mazandaran province, Iran (n = 1959). In this diagnostic research, the index test was ferritin levels measured by a chemiluminescent immunoassay. As a reference test, T2*-weighted magnetic resonance imaging (T2*-weighted MRI) was applied to determine cardiac and liver hemosiderosis. A cutoff point of 2027 ng/mL for ferritin showed a sensitivity of 50%, specificity 77.4%, PPV 42.1%, and NPV 82.5% for cardiac hemosiderosis (area under curve [AUC] 0.66, 95% CI 0.60–0.71, adjusted odds ratio [OR] 2.05, 95% CI 1.05–4.01). At an optimum cutoff point of 1090 ng/mL, sensitivity 66.7%, specificity 68%, PPV 82.9%, and NPV 46.8% for liver hemosiderosis were estimated (AUC 0.68, 95% CI 0.63–0.73, adjusted OR 3.93, 95% CI 2.02–7.64. The likelihood of cardiac hemosiderosis serum ferritin levels below 2027 ng/mL is 17.5%. Moreover, 82.9% of β-thalassemia patients with serum ferritin levels above 1090 ng/mL may suffer from liver hemosiderosis, regardless of the grades.

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“…Ferritin test is a non-speci c test providing different sensitivities and speci cities at dissimilar cutoff points for evaluating cardiac and liver hemosiderosis in multiple studies. Clinical sequelae, such as heart failure, cardiac dysrhythmia, and pulmonary hypertension from transfusional iron overload, still prevail in β-thalassemia patients 6,13,14 . In contrast, no consensus exists on the optimum cutoff of ferritin to screen for cardiac and liver hemosiderosis.…”

Section: Introductionmentioning

confidence: 99%

Ferritin Thresholds for Cardiac and Liver Hemosiderosis in β-thalassemia Patients: A Diagnostic Accuracy Study

Darvishi‐Khezri

Aliasgharian

Naderisorki

et al. 2022

Preprint

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Background and Aim: Ferritin is frequently used to screen some dire consequences of iron overload in β-thalassemia patients. The study aimed to define the best cutoff point of ferritin to screen for cardiac and liver hemosiderosis in these cases.Methods: This was a registry-based study on β-thalassemia patients living throughout Mazandaran province, Iran (n=1959). In this diagnostic research, the index test was ferritin levels measured by a chemiluminescent immunoassay. As a reference test, T2*-weighted magnetic resonance imaging (T2*-weighted MRI) was applied to determine cardiac and liver hemosiderosis.Results: A cutoff point of 2027 ng/mL for ferritin showed a sensitivity of 50%, specificity 77.4%, PPV 42.1%, and NPV 82.5% for cardiac hemosiderosis (area under curve [AUC] 0.66, 95% CI 0.60–0.71, adjusted odds ratio [OR] 2.05, 95% CI 1.05–4.01). At an optimum cutoff point of 1090 ng/mL, sensitivity 66.7%, specificity 68%, PPV 82.9%, and NPV 46.8% for liver hemosiderosis were estimated (AUC 0.68, 95% CI 0.63–0.73, adjusted OR 3.93, 95% CI 2.02–7.64. Conclusion: The likelihood of cardiac hemosiderosis serum ferritin levels below 2027 ng/mL is 17.5%. Moreover, 82.9% of ꞵ-thalassemia patients with serum ferritin levels above 1090 ng/mL may suffer from liver hemosiderosis, regardless of the grades.

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Right ventricular function in β-thalassemia children: comparing three-dimensional echocardiography with other functional parameters

Cited by 5 publications

References 31 publications

Evaluation of Right Ventricular Function in Patients with Propionic Acidemia—A Cross-Sectional Study

Evaluation of Right Ventricular Function in Patients with Propionic Acidemia—A Cross-Sectional Study

Ferritin thresholds for cardiac and liver hemosiderosis in β-thalassemia patients: a diagnostic accuracy study

Ferritin Thresholds for Cardiac and Liver Hemosiderosis in β-thalassemia Patients: A Diagnostic Accuracy Study

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