Cardiac Magnetic Resonance Strain in Beta Thalassemia Major Correlates with Cardiac Iron Overload
Deidra Ansah,
Nazia Husain,
Alexander Ruh,
Haben Berhane,
Anthony Smith,
Alexis Thompson,
Andrew De Freitas,
Cynthia K. Rigsby,
Joshua D. Robinson
Affiliations
Deidra Ansah
Department of Pediatrics, Texas Children’s Hospital at Baylor College of Medicine, Houston, TX 77030, USA
Nazia Husain
Department of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
Alexander Ruh
Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
Haben Berhane
Department of Medical Imaging, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
Anthony Smith
Department of Medical Imaging, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
Alexis Thompson
Department of Pediatrics, Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA
Andrew De Freitas
Department of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
Cynthia K. Rigsby
Department of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
Joshua D. Robinson
Department of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
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.
