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Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload

Background: Beta thalassemia major (Beta-TM) is an inherited condition which presents at around two years of life. Patients with Beta-;TM may develop cardiac iron toxicity secondary to transfusion dependence. Cardiovascular magnetic resonance (CMR) T2*, a technique designed to quantify myocardial ir...

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Autores principales: Ansah, Deidra, Husain, Nazia, Ruh, Alexander, Berhane, Haben, Smith, Anthony, Thompson, Alexis, De Freitas, Andrew, Rigsby, Cynthia K., Robinson, Joshua D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9955453/
https://www.ncbi.nlm.nih.gov/pubmed/36832400
http://dx.doi.org/10.3390/children10020271
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author Ansah, Deidra
Husain, Nazia
Ruh, Alexander
Berhane, Haben
Smith, Anthony
Thompson, Alexis
De Freitas, Andrew
Rigsby, Cynthia K.
Robinson, Joshua D.
author_facet Ansah, Deidra
Husain, Nazia
Ruh, Alexander
Berhane, Haben
Smith, Anthony
Thompson, Alexis
De Freitas, Andrew
Rigsby, Cynthia K.
Robinson, Joshua D.
author_sort Ansah, Deidra
collection PubMed
description Background: Beta thalassemia major (Beta-TM) is an inherited condition which presents at around two years of life. Patients with Beta-;TM may develop cardiac iron toxicity secondary to transfusion dependence. Cardiovascular magnetic resonance (CMR) T2*, a technique designed to quantify myocardial iron deposition, is a driving component of disease management. A decreased T2* value represents increasing cardiac iron overload. The clinical manifestation is a decline in ejection fraction (EF). However, there may be early subclinical changes in cardiac function that are not detected by changes in EF. CMR-derived strain assesses myocardial dysfunction prior to decline in EF. Our primary aim was to assess the correlation between CMR strain and T2* in the Beta-TM population. Methods: Circumferential and longitudinal strain was analyzed. Pearson’s correlation was calculated for T2* values and strain in the Beta-TM population. Results: We identified 49 patients and 18 controls. Patients with severe disease (low T2*) were found to have decreased global circumferential strain (GCS) in comparison to other T2* groups. A correlation was identified between GCS and T2* (r = 0.5; p < 0.01). Conclusion: CMR-derived strain can be a clinically useful tool to predict early myocardial dysfunction in Beta-TM.
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spelling pubmed-99554532023-02-25 Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload Ansah, Deidra Husain, Nazia Ruh, Alexander Berhane, Haben Smith, Anthony Thompson, Alexis De Freitas, Andrew Rigsby, Cynthia K. Robinson, Joshua D. Children (Basel) Article Background: Beta thalassemia major (Beta-TM) is an inherited condition which presents at around two years of life. Patients with Beta-;TM may develop cardiac iron toxicity secondary to transfusion dependence. Cardiovascular magnetic resonance (CMR) T2*, a technique designed to quantify myocardial iron deposition, is a driving component of disease management. A decreased T2* value represents increasing cardiac iron overload. The clinical manifestation is a decline in ejection fraction (EF). However, there may be early subclinical changes in cardiac function that are not detected by changes in EF. CMR-derived strain assesses myocardial dysfunction prior to decline in EF. Our primary aim was to assess the correlation between CMR strain and T2* in the Beta-TM population. Methods: Circumferential and longitudinal strain was analyzed. Pearson’s correlation was calculated for T2* values and strain in the Beta-TM population. Results: We identified 49 patients and 18 controls. Patients with severe disease (low T2*) were found to have decreased global circumferential strain (GCS) in comparison to other T2* groups. A correlation was identified between GCS and T2* (r = 0.5; p < 0.01). Conclusion: CMR-derived strain can be a clinically useful tool to predict early myocardial dysfunction in Beta-TM. MDPI 2023-01-31 /pmc/articles/PMC9955453/ /pubmed/36832400 http://dx.doi.org/10.3390/children10020271 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Ansah, Deidra
Husain, Nazia
Ruh, Alexander
Berhane, Haben
Smith, Anthony
Thompson, Alexis
De Freitas, Andrew
Rigsby, Cynthia K.
Robinson, Joshua D.
Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload
title Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload
title_full Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload
title_fullStr Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload
title_full_unstemmed Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload
title_short Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload
title_sort cardiac magnetic resonance strain in beta thalassemia major correlates with cardiac iron overload
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9955453/
https://www.ncbi.nlm.nih.gov/pubmed/36832400
http://dx.doi.org/10.3390/children10020271
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