Key result
Beta-thalassemia is linked to worsened MPI and increased PAPs despite normal T2 MRI.
Why the study?
Iron Overload Cardiomyopathy remains the main cause of death in Thalassemia major patients and early risk stratification before heart failure signs is important due to limitations of MRI T2 and conventional echocardiography.
Does Tissue Doppler Imaging (TDI) derived myocardial performance index (MPI) detect early myocardial dysfunction in beta-thalassemia patients with normal MRI T2?
Cohort (n=46)
Does Tissue Doppler Imaging (TDI) derived myocardial performance index (MPI) detect early myocardial dysfunction in beta-thalassemia patients with normal MRI T2?
TDI and MPI can detect early myocardial dysfunction in beta-thalassemia patients before changes are evident on MRI T2.
TDI-MPI may flag early dysfunction in beta-thalassemia despite normal T2; hypothesis-generating and requires prospective validation before practice change.
Background Iron Overload Cardiomyopathy (IOC) due to repeated transfusions still represents the main cause of death in Thalassemia major (TM) patients. Because iron overload remains asymptomatic for long time, it is important to stratify the patients based on the risk of developing IOC before the appearance of clinical signs of heart failure. The magnetic resonance imaging (MRI) T2* may be useful but it is expensive and its MRI software has limited availability; conventional echocardiographic parameters, although easy availability, remain normal until advanced stages of IOC. Tissue Doppler Imaging (TDI) opened a new way to explore systolic and diastolic function directly or through derived index such as myocardial performance index (MPI) which has a prognostic value in different cardiomyopathies. Methods We enrolled 46 consecutive β-TM patients without clinical signs of heart failure and we tested them with echocardiography in 2011 and again in 2015. MPI of left and right ventricular lateral wall was calculated by TDI. All TM patients had a T2* MRI evaluation of the heart in the year before. Results Despite the absence of significant changes in morphological data at echo and at T2* evaluation, S′ waves of the lateral ventricular wall decrease while time passed, MPI worsened during the follow-up period and the derived systolic pulmonary artery systolic pressure (PAPs) increased in 2015 with respect to 2011. Conclusion TDI and MPI may reveal very early myocardial dysfunction in TM patients despite a normal T2* value. Together with PAPs they should be periodically checked in these patients.
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Barbero et al. (2016) conducted a cohort in beta-Thalassemia major (n=46). Time (4-year follow-up) vs. Baseline (2011) was evaluated on Changes in S′ waves, myocardial performance index (MPI), and derived systolic pulmonary artery systolic pressure (PAPs). Myocardial performance index worsened and pulmonary artery systolic pressure increased over a 4-year follow-up in 46 beta-thalassemia patients despite normal T2 MRI.
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