Quantification of myocardial iron deposition by two-dimensional speckle tracking in patients with β-thalassaemia major and Blackfan-Diamond anaemia

P. Garceau, E. T. Nguyen, S. Carasso, H. Ross, J. Pendergrast, G. Moravsky, B. Bruchal-Garbicz, Harry Rakowski

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Cardiac disease related to transfusional iron overload is the leading cause of death in patients with β-thalassaemia major. Early myocardial iron deposition predates decreased left ventricular dysfunction and currently is best assessed by cardiac magnetic resonance. Methods: Echocardiographic speckle tracking-derived myocardial mechanics were compared with cardiac MRI T2 star (T2*) calculations in 45 chronically transfused patients with β-thalassaemia major or Diamond - Blackfan anaemia (26 retrospectively and an additional 19 for validation). Two groups were studied: patients with presumed cardiac iron overload and interventricular T2* value ≤20 ms (low T2*) and patients with >20 ms (normal T2*). They were compared with a normal control group of 18 age- and gender-matched patients. Results: Patients with low T2* had a uniform decrease in longitudinal and circumferential strain compared with normal controls (-16±3% vs -20±3% and -20±4% vs -23±5%, respectively; p<0.0005). Peak twist and peak apical rotation were lower in patients with low T2* than in those with normal T2* or normal control patients. Conversely, no significant difference was observed between patients with normal T2* and controls. There was a strong and direct logarithmic correlation between average global longitudinal strain and T2* values (r=-0.68, p=0.0007). Using a cut-off of ≤-17%, global longitudinal strain predicted a T2* value of <20 ms with a sensitivity of 76% and a specificity of 88%. Conclusion: Myocardial mechanics offers a simple alternative to cardiac MRI for assessing significant myocardial iron deposition.

Original languageEnglish
Pages (from-to)388-393
Number of pages6
JournalHeart
Volume97
Issue number5
DOIs
StatePublished - Mar 2011
Externally publishedYes

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