Role of myocardial deformation imaging in transfusion-dependent thalassemia—Correlation with severity of myocardial siderosis

Sruti Rao, Babar S. Hasan, Zahra Hoodbhoy, Ibrahim Habib, Shazia Mohsin, Rebekah Tomredle, Salima Ashiqali, Fateh A. Tipoo, Mark J. Cartoski, Bart Bijnens, Devyani Chowdhury

Research output: Contribution to journalArticlepeer-review


Objectives: To study clinical and echocardiographic parameters in Transfusion Dependent Thalassemia (TDT) patients, correlating them with T2*CMRI. To identify features of Speckle Tracking Echocardiography (STE) in those with severe TDT to prioritize patients in need of early T2*CMRI and aggressive chelation. Background: Hemosiderosis from life-long transfusions in TDT is amplified in Low- and Middle-Income Countries (LMIC) when access to resources may be scarce, delaying timely chelation. T2* imaging is the gold standard to non-invasively estimate myocardial iron and guide therapy. In resource constraint areas, we propose use of STE to prioritize those with severe disease to obtain early T2* CMRI. Methods: TDT patients (N = 25) who underwent clinical, echocardiographic, and CMRI evaluation were included, with recruitment of age matched controls. Echocardiographic indices of ventricular function and strain were correlated with T2*. Severe disease was defined as T2* < 10 ms. Differences among patients with severe disease and controls were analyzed in addition to differences between those with normal and abnormal T2*. Results: There was moderate (R −0.60, p = 0.002) inverse correlation between T2* and peak systolic global longitudinal strain (GLS) and regional basal strain (R −0.62, p < 0.001) indicating these parameters were lower in patients with severe disease when compared with controls and non-severe TDT group. GLS and most indices of regional strain were lower in patients with severe disease (T2* < 10 ms) when compared with the control group (p < 0.05). The Apex- base ratio reached significance (p = 0.02) in patients with abnormal T2*(<20 ms) when compared with normal T2*(<20 ms) in TDT positive patients. Conclusions: Early decrease in global and regional strain can serve as a useful tool to identify patients with severe TDT before reduction in ejection fraction. This study makes a case for utilizing STE as an adjunct or initial screen to T2* CMRI in LMIC to prioritize patients in need of T2*CMRI to facilitate aggressive iron chelation protocols.

Original languageEnglish
Article number101607
JournalProgress in Pediatric Cardiology
Publication statusPublished - Mar 2023


  • Chelation
  • FT CMR
  • Heart failure
  • Strain imaging
  • T2* CMRI
  • Transfusion dependent thalassemia


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