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

Author:

Ansah Deidra1,Husain Nazia2,Ruh Alexander3,Berhane Haben4,Smith Anthony4,Thompson Alexis5,De Freitas Andrew2,Rigsby Cynthia K.234ORCID,Robinson Joshua D.23ORCID

Affiliation:

1. Department of Pediatrics, Texas Children’s Hospital at Baylor College of Medicine, Houston, TX 77030, USA

2. Department of Pediatrics, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA

3. Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA

4. Department of Medical Imaging, Ann & Robert H. Lurie’s Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA

5. Department of Pediatrics, Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA

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.

Publisher

MDPI AG

Subject

Pediatrics, Perinatology and Child Health

Reference35 articles.

1. Beta-thalassemia cardiomyopathy: History, present considerations, and future perspectives;Kremastinos;Circ. Heart Fail.,2010

2. Cappellini, M.D., Cohen, A., Eleftheriou, A., Piga, A., Porter, J., and Taher, A. (2008). Guidelines for the Clinical Management of Thalassaemia, Thalassaemia International Federation.

3. Survival and causes of death in thalassaemia major;Zurlo;Lancet,1989

4. Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance;Modell;J. Cardiovasc. Magn. Reson.,2008

5. Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload;Anderson;Eur. Heart J.,2001

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