Abstract Background Beta thalassemia is an autosomal recessive disorder characterised by reduced synthesis of beta globin chains. Magnetic resonance T2* is considered the gold standard to assess cardiac iron overload and to guide iron chelation. However, some patients still present higher incidence of cardiovascular events, despite normal T2* levels. This discrepancy suggests that we might need additional parameters to identify those patients at higher risk of future events. Purpose this study aimed to assess differences in speckle tracking echocardiography (STE) parameters between transfusion dependent β-thalassemia (TDβT) patients with and without CV disease. Methods "Atrial fibrillation in β-thalassemia" is a prospective, single-center, observational study aimed at identifying the clinical, electrocardiographic and imaging features of TDβT patients. For the present cross-sectional study, global longitudinal strain (GLS), peak atrial longitudinal strain (PALS) and myocardial work (MW) were performed. The primary endpoint was the difference in STE indices between TDβT patients with and without CV disease (defined as heart failure, stroke or atherosclerotic vascular disease). The association between STE indices and CV disease was calculated using uni- and multi-variated logistic regression models. Receiver-operating characteristics (ROC) curves were constructed to identify STE parameters that could have a stronger association with CV disease and the best cut-off points for those parameters. Results between August 2022 and January 2025, 228 patients with TDβT were enrolled. Among them, 17 had a previous cardiovascular event. Patients with CV disease had higher prevalence of arterial hypertension (29% vs 9%, p-value 0.006) and atrial fibrillation (35% vs 12%, p-value 0.007). No differences were found in CMR parameters. Patients with CV disease had lower values of GLS (-19% vs -21%, p-value 0.009), PALS (25% vs 35% p-value 0.003), global constructive work (GCW - 1870 mmHg% vs 2185 mmHg%, p-value 0.004) and global work index (GWI - 1690 mmHg% vs 1952 mmHg%, p-value 0.01). ROC curve analysis revealed good diagnostic accuracy of STE indices in identify patients with CV disease and different empirical cut-off values were established for each parameter (best cut-off points: GLS -19.65%, PALS 37.1%, GWI 1966.5 mmHg%, GCW 2161 mmHg%). The highest diagnostic accuracy was achieved combining all STE indices together (sensitivity of 93%, specificity of 55%, AUC 0.77). After multivariate logistic regression analysis, GLS -19.65% (OR 3.29, 95%, CI 1.07-10.06, p-value 0.03), GWI (OR 0.99, 95% CI 0.996-0.999, p value 0.01), GCW (OR 0.99, 95% CI 0.996-0.999, p-value 0,007) and PALS (OR 0.95, 95% CI 0.90-0.99, p-value 0.04) were found to be independently associated with the presence of CV events. Conclusions in TDβ-thalassemia, patients with CV had lower values of GLS, MW and PALS. The best diagnostic accuracy was achieved combing together all STE parameters.
Marchetti et al. (Thu,) studied this question.