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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.
Affiliation
  • Ansah D; Department of Pediatrics, Texas Children's Hospital at Baylor College of Medicine, Houston, TX 77030, USA.
  • Husain N; Department of Pediatrics, Ann & Robert H. Lurie's Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
  • Ruh A; Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
  • Berhane H; Department of Medical Imaging, Ann & Robert H. Lurie's Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
  • Smith A; Department of Medical Imaging, Ann & Robert H. Lurie's Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
  • Thompson A; Department of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.
  • De Freitas A; Department of Pediatrics, Ann & Robert H. Lurie's Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
  • Rigsby CK; Department of Pediatrics, Ann & Robert H. Lurie's Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
  • Robinson JD; Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA.
Children (Basel) ; 10(2)2023 Jan 31.
Article in En | MEDLINE | ID: mdl-36832400
ABSTRACT

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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