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

BackgroundCardiac 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.MethodsEchocardiographic 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.ResultsPatients 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