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Heart disease is among the primary causes of morbidity and mortality in β-thalassemia major (β-TM). Conventional echocardiography has failed to identify myocardial dysfunction at an early stage among these patients, thus speckle tracking echocardiography (STE) has been lately used. The objectives of this review were to 1) identify all published studies having evaluated myocardial strain among β-TM patients, 2) gather their results, 3) compare their findings and 4) propose recommendations based on these data. Literature search was conducted in PubMed, SCOPUS and Cohrane Library. Data regarding left ventricular global longitudinal (LV-GLS), circumferential (LV-GCS) and radial strain (LV-GRS), right ventricular longitudinal strain (RV-GLS), left and right atrial strain were extracted. Thirty-five studies (34 original articles and 1 meta-analysis) have met the inclusion criteria. LV-GLS has been reported being worse in patients compared to controls in 13 of 21 studies, LV-GCS in 7 of 11 studies, LV-GRS in 6 of 7 studies, RV-GLS in 2 of 3 studies and left atrial strain in all case–control studies. Myocardial iron overload (MIO) patient subgroups had worse LV-GLS in 6 of 15 studies, LV-GCS in 2 of 7 studies and LV-GRS in none of 7 studies. A small number of studies suggest left atrial strain correlation with electrical atrial ectopy and atrial fibrillation. It is suggested that STE should be applied supplementary to conventional echocardiography for early identification of myocardial dysfunction among β-TM patients. Potential myocardial strain utilities could be screening for myocardial iron overload, left ventricular diastolic dysfunction and atrial fibrillation.
Heart disease is among the primary causes of morbidity and mortality in β-thalassemia major (β-TM). Conventional echocardiography has failed to identify myocardial dysfunction at an early stage among these patients, thus speckle tracking echocardiography (STE) has been lately used. The objectives of this review were to 1) identify all published studies having evaluated myocardial strain among β-TM patients, 2) gather their results, 3) compare their findings and 4) propose recommendations based on these data. Literature search was conducted in PubMed, SCOPUS and Cohrane Library. Data regarding left ventricular global longitudinal (LV-GLS), circumferential (LV-GCS) and radial strain (LV-GRS), right ventricular longitudinal strain (RV-GLS), left and right atrial strain were extracted. Thirty-five studies (34 original articles and 1 meta-analysis) have met the inclusion criteria. LV-GLS has been reported being worse in patients compared to controls in 13 of 21 studies, LV-GCS in 7 of 11 studies, LV-GRS in 6 of 7 studies, RV-GLS in 2 of 3 studies and left atrial strain in all case–control studies. Myocardial iron overload (MIO) patient subgroups had worse LV-GLS in 6 of 15 studies, LV-GCS in 2 of 7 studies and LV-GRS in none of 7 studies. A small number of studies suggest left atrial strain correlation with electrical atrial ectopy and atrial fibrillation. It is suggested that STE should be applied supplementary to conventional echocardiography for early identification of myocardial dysfunction among β-TM patients. Potential myocardial strain utilities could be screening for myocardial iron overload, left ventricular diastolic dysfunction and atrial fibrillation.
Introduction. An assessment of the relationship between the severity of hypertrophy and changes in the myocardial strain at which systolic disfunction is detected in children with hypertrophic cardiomyopathy (HCM) is clearly essential.Aim. To assess the relationship between hypertrophy and the myocardial strain in children with hypertrophic cardiomyopathy (HCM).Materials and methods. 61 patients aged between 7 and 17 years with a primary form of HCM underwent an ultrasound examination of the heart using standard techniques. An assessment of the left ventricular systolic function performed using of-line the two-dimensional (2D) speckle-tracking mode with analysis parameters that included global and segmental longitudinal, circumferential, and radial myocardial strains. The analysis of hypertrophy of myocardial segments carried out taking into account the absolute values of the thickness of the left ventricular myocardium in systole and diastole, depending on age, in terms of standard deviation units in the population (Z-score factor).Results. A decrease in longitudinal strain below the relevant values, an increase in radial strain, and no changes in circular strain were observed when the thickness of the left ventricular myocardium increased over 2.48Z. A further decrease in radial strain was observed when myocardial thickness was over 4.24Z, and circular strain was over 3.16Z. The relationship between myocardial hypertrophy and longitudinal strain had an inverse linear relationship: the lower the strain values, the greater the thickness of the myocardium. With increasing thickness of the myocardium, the radial strain first tended to increase in a compensatory manner, but it decreased when myocardial thickness increased over 4.24Z. The circular strain, as well as longitudinal one, has an inverse linear relationship, but with longer preservation of normal values when myocardial hypertrophy increases.Conclusion. Children with HCM demonstrate various types of relationships between hypertrophy and myocardial strain, which detection is important for the assessment of the left ventricular systolic function to improve the prognosis and therapeutic approach to the disease. A comprehensive approach to the assessment of myocardial strain in children with HCM should include not only a routine identification of global strain, but also assessment of the segmental strain to detect early signs of myocardial dysfunction. Comparison of measures of various types of strain and the thickness of the left ventricular myocardium has a very important diagnostic value for understanding the degree of changes in its kinetics.
Purpose: to evaluate the clinical significance of left ventricular myocardial deformation indicators, their relationship with the degree of heart failure and left ventricular hypertrophy in children with hypertrophic cardiomyopathy (HCM). Materials and methods. 61 patients with primary genetically confirmed asymmetric HCM aged 7 to 17 years underwent a comprehensive clinical and echocardiography. Echocardiography determined indicators of left ventricular systolic function: ejection and shortening fractions, global longitudinal, circular and radial myocardial deformations and deformations rates. The assessment of myocardial hypertrophy of left ventricular segments was performed taking into account absolute values in systole and diastole depending on age, recalculated per standard deviation units in the population (Z-score factor). Results. A more pronounced degree of heart failure, a decrease in ejection and shortening fractions, and indicators of left ventricular myocardial deformation were established in all children with non-obstructive and obstructive forms of HCM and an extreme degree of myocardial hypertrophy, in contrast to the non-obstructive form of the disease with a less pronounced degree of hypertrophy. An earlier decrease in myocardial deformation indices was revealed in comparison with ejection and shortening fractions, longitudinal deformation and its speed in comparison with radial and circular, their speeds. Conclusions. 1. In children with HCM and extreme degrees of left ventricular myocardial hypertrophy, compared with mild and moderate hypertrophy, a more pronounced degree of heart failure is observed, which is accompanied by a decrease in left ventricular systolic function. Among them, a decrease in myocardial deformation rates and their velocities is more sensitive compared to left ventricular ejection fractions and shortening. 2. Reduction of left ventricular myocardial deformation in children with HCM depends on the degree of hypertrophy. The earliest, manifesting itself even with a mild degree of hypertrophy, is a decrease in longitudinal deformation and its speed compared to circular and radial deformations and their speeds. 3. Determining the indicators of left ventricular myocardial deformation in children with HCM in comparison with the degree of myocardial hypertrophy is of great clinical importance in assessing the systolic function of the left ventricle to optimize administration tactics and timely treatment of the disease.
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