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Role of myocardial deformation imaging in transfusion-dependent thalassemia---correlation with severity of myocardial siderosis
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  • Sruti Rao ,
  • Babar Sultan Hasan,
  • Zahra Hoodbhoy,
  • Mark Cartoski,
  • Rebekah Tomredle,
  • Shazia Mohsin,
  • Salima Ashiqali,
  • Ibrahim Habib,
  • Fateh Tipoo,
  • Bart Bijnens,
  • Devyani Chowdhury
Sruti Rao
Alfred I DuPont Hospital for Children
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Babar Sultan Hasan
The AGA KHAN UNIVERSITY
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Zahra Hoodbhoy
The Aga Khan University Medical College Pakistan
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Mark Cartoski
Alfred I DuPont Hospital for Children
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Rebekah Tomredle
Cardiology Care for Children
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Shazia Mohsin
The Aga Khan University Medical College Pakistan
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Salima Ashiqali
The Aga Khan University Medical College Pakistan
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Ibrahim Habib
The Aga Khan University Medical College Pakistan
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Fateh Tipoo
The Aga Khan University Medical College Pakistan
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Bart Bijnens
, Institut d’Investigacions Biomediques August Pi I Sunyer (IDIBAPS)
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Devyani Chowdhury
Cardiology Care for Children

Corresponding Author:[email protected]

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Abstract

Objectives To study clinical and echocardiographic parameters in TDT patients, correlating these indices with T2*CMRI. To identify a deformational imaging metric in LMIC with severe TDT, to allow for early T2*CMRI. Background TDT requires lifelong transfusions, increasing the risk of multi organ dysfunction from iron toxicity. The problem is amplified in LMIC where access to resources maybe scarce, delaying timely chelation. T2* imaging is the gold standard to non-invasively estimate myocardial iron, to guide therapy. In resource constraints areas, we propose the use of Speckle Tracking Echocardiography (STE) to prioritize those with severe disease to obtain early T2* CMRI. Methods Twenty-five TDT patients who had undergone clinical, echocardiographic and CMRI evaluation were included in the study. Age matched controls with only echocardiographic data were recruited. Spearmans correlation was used to correlate echocardiographic indices of ventricular function and strain with T2*. Differences among patients with severe disease i.e. T2*<10 vs the remaining TDT group and controls was analyzed using Mann-Whitney U test. Results STE showed moderate inverse correlation with T2*CMRI for GLS and various indices of regional strain. GLS and regional strain were lower in patients with severe disease (T2* <10) when compared with normal controls and the rest of the TDT positive group (T2* >10) Conclusions Early decrease in GLS and regional strain, can serve as a useful tool to stratify those patients at risk of severe iron overload before systolic dysfunction ensues, serving as an adjunct in LMIC to prioritize T2* CMRI analysis for intensification of chelation therapy.