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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Main Authors: Deidra Ansah, Nazia Husain, Alexander Ruh, Haben Berhane, Anthony Smith, Alexis Thompson, Andrew De Freitas, Cynthia K. Rigsby, Joshua D. Robinson
Format: Article
Language:English
Published: MDPI AG 2023-01-01
Series:Children
Subjects:
Online Access:https://www.mdpi.com/2227-9067/10/2/271
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author Deidra Ansah
Nazia Husain
Alexander Ruh
Haben Berhane
Anthony Smith
Alexis Thompson
Andrew De Freitas
Cynthia K. Rigsby
Joshua D. Robinson
author_facet Deidra Ansah
Nazia Husain
Alexander Ruh
Haben Berhane
Anthony Smith
Alexis Thompson
Andrew De Freitas
Cynthia K. Rigsby
Joshua D. Robinson
author_sort Deidra Ansah
collection DOAJ
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; <i>p</i> < 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 doaj.art-24c29c8c9bb64341ae28cdcb20eef7602023-11-16T19:48:25ZengMDPI AGChildren2227-90672023-01-0110227110.3390/children10020271Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron OverloadDeidra Ansah0Nazia Husain1Alexander Ruh2Haben Berhane3Anthony Smith4Alexis Thompson5Andrew De Freitas6Cynthia K. Rigsby7Joshua D. Robinson8Department of Pediatrics, Texas Children’s Hospital at Baylor College of Medicine, Houston, TX 77030, USADepartment of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USADepartment of Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USADepartment of Medical Imaging, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USADepartment of Medical Imaging, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USADepartment of Pediatrics, Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USADepartment of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USADepartment of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USADepartment of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USABackground: 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; <i>p</i> < 0.01). Conclusion: CMR-derived strain can be a clinically useful tool to predict early myocardial dysfunction in Beta-TM.https://www.mdpi.com/2227-9067/10/2/271strainbeta thalassemia majorIron OverloadIron metabolismcardiomyopathiesmagnetic resonance imaging
spellingShingle Deidra Ansah
Nazia Husain
Alexander Ruh
Haben Berhane
Anthony Smith
Alexis Thompson
Andrew De Freitas
Cynthia K. Rigsby
Joshua D. Robinson
Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload
Children
strain
beta thalassemia major
Iron Overload
Iron metabolism
cardiomyopathies
magnetic resonance imaging
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 strain
beta thalassemia major
Iron Overload
Iron metabolism
cardiomyopathies
magnetic resonance imaging
url https://www.mdpi.com/2227-9067/10/2/271
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