Systolic and diastolic function assessment in fabry disease patients using speckle-tracking imaging and comparison with conventional echocardiographic measurements.

Fabry cardiomyopathy is characterized by progressive left ventricular hypertrophy (LVH) associated with diastolic dysfunction and is the most common cause of death in Fabry disease (FD). However, LVH is not present in all subjects, particularly early in disease progression and in female patients. Direct assessment of myocardial deformation by strain and strain rate (SR) analysis may be sensitive to detect subclinical Fabry cardiomyopathy independent of the presence of LVH. Systolic (longitudinal, circumferential, and radial systolic strain and SR) and diastolic (SR during isovolumic relaxation [SR(IVR)] and early diastole and strain at peak transmitral E wave) function was assessed in 16 patients with FD using two-dimensional speckle-tracking echocardiography. In addition, mean S' and E' mitral annular velocities by Doppler tissue imaging were measured. Diastolic filling indices, including E/SR(IVR) and E/E' ratios, were calculated. The patients were compared with 24 healthy age-matched and gender-matched controls. All 16 patients with FD had normal left ventricular ejection fractions, and nine patients had LVH. Compared with controls, patients with FD had reduced longitudinal systolic strain (P < .001) and systolic SR (P = .007), while there were no differences in circumferential systolic strain and S'. Diastolic function assessment showed reduced longitudinal early diastolic SR (P = .001), SR(IVR) (P < .001), and E/SR(IVR) (P < .001), while radial and circumferential diastolic function was not affected. Of the conventional diastolic function indices, reductions were seen in E (P = .006), E' (P = .021), and E/E' ratio (P < .001). After correcting for LVH, only SR(IVR) (P < .001) and E/SR(IVR) (P = .025) remained significantly different between patients with FD and controls, with sensitivity of 94% and specificity of 92% for SR(IVR) of 0.235 sec(-1) (area under the receiver operating characteristic curve, 0.953). Strain and SR analysis is useful in identifying patients with FD with reduced myocardial function, with longitudinal systolic strain and diastolic isovolumic SR being superior to the other echocardiographic measurements of myocardial contraction and relaxation and independent of LVH.

Shanks M ，Thompson RB ，Paterson ID ，Putko B ，Khan A ，Chan A ，Becher H ，Oudit GY ... -  《-》

Left atrial enlargement and reduced atrial compliance occurs early in Fabry cardiomyopathy.

Fabry disease is associated with left ventricular hypertrophy (LVH) and myocardial fibrosis. The aim of this study was to evaluate left atrial (LA) size and function using tissue Doppler-derived strain in patients with Fabry disease. Echocardiography was performed in 33 Fabry patients (14 without LVH, 19 with LVH) before commencement of enzyme replacement therapy, and results were compared with those from age-matched and gender-matched controls (n=28 and n=38, respectively). Atrial strain and strain rate were measured from four segments in the apical four-chamber and two-chamber views of the LA, and global values were calculated. Systolic strain, systolic strain rate, early diastolic strain rate, and late diastolic strain rate were measured. Phasic LA volumes and fractions were calculated. Mitral inflow and tissue Doppler E' velocities were used to estimate left ventricular (LV) diastolic function. LA volume was increased in Fabry patients, even in the absence of LVH. Importantly, diastolic function was normal in this subgroup without LVH, with E' velocities similar to those in controls. LA systolic strain and early diastolic strain rate were selectively reduced in Fabry patients with LVH and reflect reductions in LA and LV relaxation, respectively, consequent to increased LV mass. However, independent of LVH, both Fabry groups had significant reductions in systolic strain rate and increased LA stiffness index. Fabry disease is associated with LA enlargement and reduced atrial compliance that occurs before the development of LVH. This suggests that Fabry cardiomyopathy may not only cause ventricular hypertrophy and fibrosis but also alters atrial myocardial properties early in the disease process. Consequently, measurements of LA size and function may be useful in the early diagnosis of Fabry disease, before the development of LVH.

Boyd AC ，Lo Q ，Devine K ，Tchan MC ，Sillence DO ，Sadick N ，Richards DA ，Thomas L ... -  《-》

Severe obesity impairs systolic and diastolic heart function - the significance of pulsed tissue Doppler, strain, and strain rate parameters.

We assessed the impact of morbid obesity on systolic and diastolic heart function in severely obese, but otherwise healthy subjects and age-matched controls. Overall, 27 obese patients: 19 female (F) and 8 male (M) mean age 37 ± 9, mean body mass index (BMI) 46 ± 6 and 27 control subjects: mean age 36 ± 10, mean BMI 23 ± 3 were examined by transthoracic echocardiography, including tissue Doppler echocardiographic (TDE) assessment and speckle tracking echocardiography to measure left ventricular longitudinal, circumferential, and radial strain (S) and strain rate (SR) during systolic and early diastolic phase. Obese patients presented with enlargement of both ventricles and the left atrium, and thicker left ventricular wall. Although left ventricular ejection fraction (EF) as well as amplitude of tricuspid annulus plane systolic excursion (TAPSE) was similar, the amplitude of mitral annulus plane systolic excursion (MAPSE) and ventricular systolic and early diastolic velocities measured by TDE were significantly lower for both ventricles in the obese group. (RV S' 13 ± 3 cm/sec vs. 15 ± 2 cm/sec, P = 0.0057; LV S' lat 8.5 ± 1.6 cm/sec vs. 12.1 ± 2.8, P < 0.0001, E' lat: 12.4 ± 2.9 vs. 16.4 ± 3.5 cm/sec for left ventricular and E' 12 ± 3 cm/sec vs. 18 ± 4 for right ventricular velocities, P < 0.0001). Among the deformation parameters, systolic and diastolic circumferential and systolic radial strain and SR were decreased in the obese subjects, whereas longitudinal strain did not differ significantly. TDE parameters documented reduced systolic and diastolic function of both ventricles in obese patients. 2-dimensional speckle tracking analysis revealed that circumferential and radial but not longitudinal strain and SR were impaired in the obese group.

Wierzbowska-Drabik K ，Chrzanowski L ，Kapusta A ，Uznańska-Loch B ，Płońska E ，Krzemińska-Pakuła M ，Kurpesa M ，Rechciński T ，Trzos E ，Kasprzak JD ... -  《-》

A comparison of Doppler, tissue Doppler imaging, and strain rate imaging in the assessment of postexercise left ventricular function.

Shave R ，George K ，Whyte G ，Middleton N ，Hart E ，Artis N ，Oxborough D ... -  《Applied Physiology Nutrition and Metabolism》

Differences of myocardial systolic deformation and correlates of diastolic function in competitive rowers and young hypertensives: a speckle-tracking echocardiography study.

The aim of this study was to compare speckle-tracking echocardiography-derived left ventricular (LV) systolic mechanics and their relationships with LV diastolic properties in young patients with hypertension and in young competitive athletes in relation to their respective alterations of LV structure. Nineteen sedentary controls, 22 top-level rowers, and 18 young newly diagnosed, never-treated patients with hypertension, all male, underwent Doppler echocardiography including pulsed tissue Doppler of the mitral annulus and speckle-tracking echocardiography. Peak longitudinal strain was calculated in apical long-axis, four-chamber, and two-chamber views, and values of the three views were averaged (global longitudinal strain [GLS]). Regional circumferential and radial strain were calculated at the LV basal, middle, and apical levels, and values were averaged (global circumferential strain and global radial strain). LV torsion was determined as the net difference in the mean rotation between the apical and basal levels. The three groups were comparable for age, whereas body mass index and blood pressure were higher in patients with hypertension, and heart rate was lower in rowers. LV mass index was higher in rowers and in patients with hypertension than in controls, without differences in relative wall thickness, ejection fraction, and midwall shortening. Left atrial volume index was greater in rowers than in controls and patients with hypertension. Annular systolic velocity (s') (P < .001) and early diastolic velocity (e') (P < .0001) were lower and the E/e' ratio was higher (P < .0001) in patients with hypertension. GLS was lower in patients with hypertension (-17.5 ± 2.8%) than in rowers (-22.2 ± 2.7%) and in controls (-21.1 ± 2.0%) (P < .0001). Global circumferential strain, global radial strain, and torsion were similar among the three groups. In the pooled population, GLS was an independent contributor to E/e' ratio (P < .0001) after adjusting for age, heart rate, meridional end-systolic stress, LV mass index and left atrial volume index. By receiver operating characteristic curve analyses, both GLS and E/e' ratio appeared to be accurate in discriminating patients with hypertension from healthy controls, with the E/e' ratio being more sensitive (77.8%) and GLS more specific (89.5%). The hearts of young patients with hypertension are characterized by reduced GLS, whereas global circumferential strain, global radial strain, and torsion are similar to those of athletes' hearts. The extent of GLS is strongly associated with LV diastolic function, independently of afterload changes and the degree of LV hypertrophy.

Galderisi M ，Lomoriello VS ，Santoro A ，Esposito R ，Olibet M ，Raia R ，Di Minno MN ，Guerra G ，Mele D ，Lombardi G ... -  《-》