Global Diastolic Strain Rate Research Articles

Impact of Pulmonary Hemodynamics and Ventricular Interdependence on Left Ventricular Diastolic Function in Children With Pulmonary Hypertension.

Through ventricular interdependence, pulmonary hypertension (PH) induces left ventricular (LV) dysfunction. We hypothesized that pediatric PH patients have LV diastolic dysfunction, related to adverse pulmonary hemodynamics, leftward septal shift, and prolonged right ventricular systole. Echocardiography was prospectively performed at 2 institutions in 54 pediatric PH patients during cardiac catheterization and in 54 matched controls. Diastolic LV measures including myocardial deformation were assessed by echocardiography. PH patients had evidence of LV diastolic dysfunction, most consistent with impaired LV relaxation, though some features of reduced ventricular compliance were present. PH patients demonstrated the following: reduced mitral E velocity and inflow duration, mitral E' and E'/A', septal E' and A', pulmonary vein S and D wave velocities, and LV basal global early diastolic circumferential strain rate and increased mitral E deceleration time, LV isovolumic relaxation time, mitral E/E', and pulmonary vein A wave duration. PH patients demonstrated leftward septal shift and prolonged right ventricular systole, both known to affect LV diastole. These changes were exacerbated in severe PH. There were no statistically significant differences in diastolic measures between patients with and without a shunt and minimal differences between patients with and without congenital heart disease. Multiple echocardiographic LV diastolic parameters demonstrated weak-to-moderate correlations with invasively determined PH severity, leftward septal shift, and prolonged right ventricular systole. Pediatric PH patients exhibit LV diastolic dysfunction most consistent with impaired relaxation and reduced myocardial deformation, related to invasive hemodynamics, leftward septal shift, and prolonged right ventricular systole.

Decreased regional left ventricular myocardial strain in type 1 diabetic children: a first sign of diabetic cardiomyopathy?

Type 1 diabetes is a major cardiovascular risk factor associated with an excess of mortality in young adults due to premature cardiovascular events, which includes heart failure. The relation between type 1 diabetes and cardiac structure and function in children was poorly documented. Our study investigates (1) whether type 1 diabetic children have echocardiographic signs of subclinical cardiac dysfunction assessed by tissue Doppler strain and (2) whether state of metabolic control and diabetes duration have any influence on the cardiac event. Standard echocardiography and tissue Doppler imaging were prospectively performed in type 1 diabetic children. Left ventricular dimensions, standard indices of systolic and diastolic function, and septal longitudinal strain were investigated. Thirty consecutive asymptomatic diabetic children (age: 12.4 [5-17] years; males: 53%) were compared to 30 age and sex-matched healthy control subjects. Left ventricular mass index and diastolic septal thickness were significantly increased in diabetic children. There was no difference between two groups as regards the left ventricular ejection fraction and conventional mitral Doppler parameters (E, A, Ea). The global longitudinal systolic strain and strain rate were found to be decreased in children with diabetes. The global longitudinal early diastolic strain rate (Esr) was negatively correlated with metabolic control. Longitudinal strain was not correlated with diabetes duration. Children with Type 1 diabetes had subclinical alterations in left ventricular size and longitudinal myocardial deformation.

Assessment of left ventricular function in young type 1 diabetes mellitus patients by two-dimensional speckle tracking echocardiography: Relation to duration and control of diabetes

Abstract Background The effect of type 1 diabetes mellitus (T1DM) on myocardial function is still controversial. Aim To examine the usefulness of speckle tracking echocardiography (STE) in detecting subclinical left ventricular (LV) dysfunction in asymptomatic T1DM patients and detect whether any LV abnormalities are related to duration or control of DM. Methods Sixty young T1DM patients and 30 controls were subjected to conventional echocardiography and STE. The left ventricular (LV) peak systolic (PS) global longitudinal strain (GLS)/strain rate (GLSR) and diastolic global strain rate during the isovolumic relaxation period (GSRivr), early diastole (GSRe) and late diastole (GSRa) were calculated as the average of the 12 myocardial segments of the four and two-chamber views while the mid circumferential strain (MCS) was calculated as the average of strains of the 6 LV segments of the mid LV cavity. Results In diabetic patients, the LV end diastolic dimension (LVEDD) was significantly lower ( p = 0.000) while the LV myocardial performance index (LVMPI) and E/lateral E′ ratio were significantly higher ( p = 0.000 and p = 0.02 respectively) compared to controls. By STE, only the GLS was significantly lower in diabetic patients compared to controls ( p = 0.000). A significant negative correlation was detected between LDL-C and GLS ( r = −0.4; p = 0.002). Multivariate logistic regression analysis identified HbA1c as the only independent predictor of GLS in patients (beta = −0.34, 95% CI: −0.67 to −0.19; p = 0.001). Conclusion STE is useful to detect subclinical LV dysfunction in asymptomatic patients with T1DM. Control of DM but not duration of disease was an independent predictor of LV systolic dysfunction.

Evaluation of the Right Ventricular Function in Prediabetes: A 2-D Speckle Tracking Echocardiographic Study

Pre-diabetes is a common condition associated with cardiovascular changes. The aim of our study was to evaluate the right ventricular (RV) function as assessed by 2-D speckle tracking echocardiography in pre-diabetic patients. This study recruited 94 patients (33 normal patients, 31 pre-diabetic patients and 30 diabetic patients). The absolute amount of the global peak systolic strain, the systolic strain rate and the early diastolic strain rate of the RV free wall (RVFW) was higher in the control group than in the pre-diabetic and diabetic groups. These indices were not different between the pre-diabetic and diabetic groups. The global late diastolic strain rate of RVFW was the same between the three groups. Also, pre-diabetes and diabetes were the independent predictors of the RVFW global peak systolic strain, systolic strain rate and diastolic strain rate. Our study demonstrated that the RV systolic and diastolic functions were impaired in the pre-diabetic and diabetic patients without obstructive coronary artery disease. Also, pre-diabetes and diabetes were independent predictors of systolic and diastolic functions of the RV.

Pathophysiological effects of different risk factors for heart failure

BackgroundHypertension and type 2 diabetes mellitus (T2DM) are important causes of non-ischaemic heart failure (HF). Understanding the pathophysiology of early HF may guide screening. We hypothesised that the underlying physiology...

Association of the Ratio of Early Mitral Inflow Velocity to the Global Diastolic Strain Rate with a Rapid Renal Function Decline in Atrial Fibrillation.

The ratio of early mitral inflow velocity (E) to the global diastolic strain rate (E’sr) has been correlated with left ventricular filling pressure and predicts adverse cardiac outcomes in atrial fibrillation (AF). The relationship between the E/E’sr ratio and renal outcomes in AF has not been evaluated. This study examined the ability of the E/E’sr ratio in predicting progression to the renal endpoint, which is defined as a ≥ 25% decline in the estimated glomerular filtration rate in patients with AF. Comprehensive echocardiography was performed on 149 patients with persistent AF, and E’sr was assessed from three standard apical views using the index beat method. During a median follow-up period of 2.3 years, 63 patients (42.3%) were reaching the renal endpoint. Multivariate analysis showed that an increased E/E’sr ratio (per 10 cm) (hazard ratio, 1.230; 95% confidence interval, 1.088 to 1.391; p = 0.001) was associated with an increased renal endpoint. In a direct comparison, the E/E’sr ratio outperformed the ratio of E to early diastolic mitral annular velocity (E’) in predicting progression to the renal endpoint in both univariate and multivariate models (p ≤ 0.039). Moreover, adding the E/E’sr ratio to a clinical model and echocardiographic parameters provided an additional benefit in the prediction of progression to the renal endpoint (p = 0.006). The E/E’sr ratio is a useful parameter and is stronger than the E/E’ ratio in predicting the progression to the renal endpoint, and it may offer an additional prognostic benefit over conventional clinical and echocardiographic parameters in patients with AF.

Comparison of regional and global cardiac MRI diastolic strain rates with echo grading of diastolic dysfunction

Comparison of regional and global cardiac MRI diastolic strain rates with echo grading of diastolic dysfunction

0056: Assessment of left ventricular filling pressures: a prospective speckle tracking echocardiography compared to invasive hemodynamic study

Background Assessment of left ventricular filling pressures (LVFP) is a key part of echocardiographic evaluation but actual validated parameters aren’t relevant in some routine cases. Use of diastolic strain rates (DSr) may improve the diagnostic of elevated LVFP in specific cardiomyopathies. Our primary objective was to evaluate the diagnostic performance for elevated LVFP of different DSr indexes in a heterogenous cardiological population. Material and Methods We led a prospective mono-centric two-dimensional speckle tracking echocardiography study compared to invasive hemo-dynamic (left catheterization with measurement of pre-atrial pressure (preA)). Elevation of LVFP was defined as preA>15mmHg. Global DSr indexes tested were: isovolumetric relaxation DSr (IvrDSr), early DSr (EarlyDSr) and late DSr (LateDSr). Additional DSr indexes were calculated by meaning of these parameters: EarlyLateDSr and IvrEarlyLateDSr. DSr values were multiplied by 10 factor and ratios of E wave to the obtained result gave different composite E/10DSr indexes. Results Fifty height patients were included. Patients with preA>15mmHg (n=23) were compared with patients with preA 52% of patients had coronaropathy, 43% had regional dysfunction and 31% had significant valvulopathy. Correlations to LVFP were: E/10IvrEarlyLateDSr (R=0,50), E/10LateDSr (R=0,46), E/10EarlyLateDSr (R=0,42). Optimal cut off values were 11,5 for E/10LateDSr (sensitivity (Se) 64%, specificity (Spe) 91%, positive predictive value (PPV) 82% and negative predictive value (NPV) 80%), 9,5 for E/10EarlyLateDSr (Se 68%, Spe 83%, PPV 71%, NPV 81%) and 15 for E/10IvrEarlyLateDSr (Se 75%, Spe 85%, PPV 79%, NVP 82%). Conclusion E/10DSr composite indexes, especially E/10LateDSr and E/ 10IvrEarlyLateDSr are relevant LVFP indexes, which may be applied to a large spectrum of cardiomyopathies. Download : Download high-res image (135KB) Download : Download full-size image Abstract 0056 - Figure: Used DSr

Relation between Left Atrial Remodeling in Young Patients with Cryptogenic Stroke and Normal Inter-atrial Anatomy.

Background and PurposeTo investigate an association between left atrial (LA) structural and P wave dispersion (PWD) during sinus rhythm, and electrical remodeling in cryptogenic stroke (CS) patients.MethodsForty CS patients and 40 age- and sex-matched healthy controls were enrolled. P wave calculations were based on 12-lead electrocardiography (ECG) at a 50-mm/s-paper speed with an amplitude of 10 mm/mV. Difference between the maximum and minimum P wave duration was the P wave dispersion (PWD=Pmax-Pmin). LA deformation was evaluated by speckle tracking echocardiography within 3 days of the acute event.ResultsPWD was 30.1±7.0 ms and 27.4±3.5 ms in CS and control group (P=0.02), whereas LA maximum volume index [LAVImax] was 20.4±4.5 mL/m2 and 19.9±2.4 mL/m2 in CS and control group, respectively (P = 0.04). While global peak LA strain was [pLA-S] (LA reservoir function) 41.4 ± 6.3% and 44.5 ± 7.1% in CS and control group, (P = 0.04), global peak late diastolic strain rate values [pLA-SRa] (LA pump function) were 2.5 ± 0.4% and 2.9 ± 0.5% in CS and control group, respectively (P = 0.001). A mild and a strong negative correlation between global pLA-S and LAVImax (r=-0.49; P<0.01), and between PWD and global pLA-S (r = -0.52; P < 0.01), respectively, was observed in CS.ConclusionsIncreased PWD is associated with impaired LA mechanical functions and enlargement, and involved in the pathophysiology of AF or an AF-like physiology in CS.

Early Changes in 2D-Speckle-Tracking Echocardiography May Predict a Decrease in Left Ventricular Ejection Fraction in Lymphoma Patients Undergoing Anthracycline Chemotherapy: A Pilot Study

Background: Two-dimensional speckle-tracking echocardiography (2D-STE) may detect early changes in cardiac mechanics in patients with normal left ventricular ejection fraction (LVEF). We aimed to evaluate whether changes in strain or strain rate, as measured by 2D-STE, precede a decrease in LVEF in patients with lymphoma undergoing anthracycline-based chemotherapy (AC). Methods: We studied patients with lymphoma who underwent 2D-STE before and after starting AC. A cardiotoxicity “event” was defined as a decrease in LVEF by ≥10% or to <50%. Standard echocardiographic and strain indices were analyzed. Results: A total of 46 patients with lymphoma (54% men; mean (SD) age, 64 (14) years) were included. An event occurred in 12 patients after starting AC. Abnormalities were detected in global longitudinal strain (GLS), peak systolic global longitudinal strain rate (GLSRs), and global circumferential strain rate (GCSR) at <6 months and in global longitudinal early diastolic strain rate (GLSRe) at 6-12 months before a decrease in LVEF (GLS odds ratio (OR) (95% CI) 1.86 (1.28-2.91), P<0.001; global circumferential strain OR (95% CI) 1.47(1.13-1.97), P=0.005; GLSRs OR (95% CI) 1.31 (1.10-1.61), P=0.002; and GCSR OR (95% CI) 1.08 (1.00-1.19), P=0.0495). Conclusions: GLS and GLSRs were the earliest and most predictive echocardiographic strain parameters for AC-induced cardiomyopathy in this pilot study. These parameters may be incorporated into cardiotoxicity surveillance protocols for patients undergoing AC.

Predictors of Functional Capacity in Patients with Pulmonary Hypertension

Introduction: The 6-minute walk test (6 MWT) distance is frequently used in the prediction of pulmonary hypertension (PH) prognosis. However, potential surrogates of this measure have not been established. We aim to describe the clinical, echocardiographic, and laboratorial criteria determining the 6 MWT distance in patients with PH. Methods: In 22 consecutive PH patients, functional capacity was evaluated by the 6 MWT distance and compared with levels of neurohormonal activation biomarkers and echocardiographic parameters for right ventricular (RV) function. Results: A correlation between the 6 MWT distance and several clinical parameters was found: Borg’s pre-test classification (R=-0.46; p=0.038); WHO functional class (p=0.029). Patients with higher levels of aldosterone (R=-0.46; p=0.030), renin (R=-0.43; p=0.046) and mid-regional pro-adrenomedullin (MR-proADM, R=-0.53; p=0.009) had worse 6 MWT performance. The opposite was true for natremia levels (R=0.55; p=0.006). There was a positive correlation between the 6 MWT distance and RV global longitudinal peak systolic strain rate and early diastolic strain rate (R=0.49; p=0.038 and R=-0.55; p=0.018, respectively). On multivariate analysis, only natremia and early diastolic strain rate were predictors of the 6 MWT distance. Conclusion: The 6 MWT distance correlated with renin-angiotensin-aldosterone system activation and parameters for RV myocardial deformation. The new biomarker MR-proADM proved to be useful in the prediction of the functional capacity.

0258: Prognostic value of new diastolic parameter derived from a two-dimensional speckle-tracking study in acute myocardial infarction

Diastolic dysfunction in acute myocardial infarction (MI) reflect severe damage and adverse outcome spatially in diabetics patients. Recently, the ratio of early mitral inflow velocity (E) to global diastolic strain rate (e’sr) has been proposed as a marker of elevated LV filling pressure and perhaps a prognostic parameter. The aim of this study to evaluate the E/e’sr ratio in diabetics and its association with adverse outcome in patients with MI. We prospectively included patients with MI and performed echocardiography with comprehensive diastolic evaluation including E/e’sr. 52% were diabetics. We compared the relationship between E/e’sr and the primary composite endpoint (all-cause mortality, hospitalization for heart failure (HF), stroke) in the 2 groups and with Cox models analysis. A total of 458 patients (mean age 59±12, 62% male) were included and 63 patients (13.7%) reached the primary endpoint (median follow-up 13 months). A significant prognostic value was found for E/e’sr [hazard ratio (HR) per 1unit change: 2.56, 95% confidence interval (CI): 1.2-4.55, p<0.0001] in diabetics group. After multivariable adjustment E/e’sr remained independently related to the combined endpoint (HR per 1 unit change, 1.370; CI: 1.01-6.33, P = 0.03). The prognostic value of E/e’sr was correlated to left ventricular ejection fraction on admission, < 35% (r=0.678, p=0.0002)and C reactive protein value, >40 mg/l, (r=0.589, p=0.001). Deformation-based E/e’sr contributes important information about global myocardial relaxation superior to velocity-based analysis in diabetic patients and correlated with left ventricular function and inflammatory status an is independently associated with the outcome in acute MI

Abstract 15891: Age-Related Changes of Right Atrial and Right Ventricular Functions Assessed by Speckle Tracking Echocardiography

Introduction: Limited data are reported about age-related changes of the RA phasic functions, assessed by speckle tracking echocardiography (STE), and their correlation with the RV function. Methods: 131 normal subjects (19-78 yrs, 89 women), divided in 4 age groups, were studied by conventional, tissue Doppler, and STE. Active RA function was assessed from negative global strain at atrial contraction (GSA-) and derived time from P onset (TGSA-), and late diastolic SR (GSRL); passive function from positive global strain at TVO (GSA+) and time from R to peak (TGSA+), and from early negative diastolic global strain rate (GSRE); reservoir function from the sum of GSA- and GSA+ (SUMGSA), positive global SR at the beginning of RV systole (GSR+) and derived time from P onset (TGSR+). RA volumes were measured. RV systolic function was assessed from TAPSE, FAC, S’, global systolic strain (GS) and strain rate (RVSRs); diastolic function from early and late global SR (RVSRe and RVSRl). Results: Active RA function was not affected by age, but RA contraction time was longer over 50 yrs. Reservoir function decreased starting from 50 yrs (GRS+), but especially over 60 yrs (SUMGSA, TGRS+). Passive function also decreased over 50 yrs (GSA+, TGSA+, GSRE). RV systolic function was not affected by age, whereas RVSRe decreased, and RVSRl increased over 50 yrs (Table). Global RV function (TAPSE, FAC) did not correlate with any of the RA functions. However, longitudinal RV systolic function (RVSRs, GS, S’) correlated inversely with active function (GSA-, GSRL). RVSRe had positive correlation with RA reservoir (SUMGSA, GSR+), and also with passive function (GSA+, GSRE) (r=0.4, p&lt;0.001). Atrial volumes were not affected by age. Conclusion: Active RA function is not affected by age. Passive and reservoir RA functions, and diastolic RV function decreased over 50 yrs. Longitudinal systolic RV correlates with active RA function, and diastolic RV function with passive and reservoir RA functions.

Comparison of magnetic resonance feature tracking with harmonic phase imaging analysis (CSPAMM) for assessment of global and regional diastolic function

AimsComplex post-processing is required for strain-derived assessment of diastolic dysfunction (DD) using CMR-tagging (TAG). Feature-tracking (FT), allows for rapid systolic strain assessment using conventional steady-state free precession (SSFP)-Cine sequences. Aim of this study was to investigate whether FT may be employed for the clinically applicable quantification of DD. Methods and Results40 individuals (20 patients with DD I-III°, 20 controls) were investigated. CSPAMM and SSFP-Cine sequences were acquired in identical short-axis locations. Global and regional early diastolic strain rate (EDSR), peak diastolic strain rate (PDSR), twist, untwist and torsion were calculated from tagged and SSFP-Cine datasets. DD indices were compared, intra- as well inter-observer variability assessed. Resultsfor global EDSR correlated strongly (r=0.94), revealed good agreement and no significant differences between both methods. Correlation for regional EDSR was lower, results differed significantly in the anterior wall (p<0.05). Correlation for PDSR was moderate (r=0.63), results in the healthy control group differed significantly (p<0.05). FT derived rotational indices correlated poorly with TAG (twist: r=0.28; untwist: r=0.02; torsion: r=0.26), subgroup analysis revealed significant differences (p<0.05). Intra- and inter-observer variability for FT derived global EDSR and PDSR were comparable to TAG, but significantly higher for regional EDSR and rotational indices. ConclusionFT derived global EDSR allows for rapid clinical determination of diastolic dysfunction, revealing good agreement with TAG and low intra- as well as interobserver variability. However, TAG analysis not only yields higher accuracy and reproducibility of global- and regional diastolic strain, but also delivers reliable information about diastolic rotational and untwisting dynamics.

Coronary calcification with no flow limiting lesions: A potential cause for ischaemic dysfunction in syndrome X patients

AimExertional angina in patients with no coronary flow limiting lesions remains a clinical puzzle. We aimed to assess the extent of coronary artery calcification (CAC) and its relationship to ventricular wall motion function using stress echocardiography in a group of patients limited by exertional angina, but no obstructive lesions. MethodsWe compared CT coronary calcium score (CACS) and dobutamine stress echocardiography in 55 patients (age 64.7±7.7years), divided into Group 1 (CACS≤100) and Group 2 (CACS>100). No patient had LV ejection fraction-EF<55%, pulmonary hypertension, arrhythmia, renal failure or parathyroid disease. Multiple linear regression analysis was used to test the association between gender-standardized continuous echocardiographic parameters and patient groups adjusted for age, body surface area, osteoporosis and CV risk factors and CACS. ResultsAt rest, LV long axis ‘subendocardial’ function was reduced (amplitude: β −1.11 SD, p<0.05, R2 0.6 and systolic velocity: β −1.08 SD, p<0.05, R2 0.44), left atrial (LA) indexed volume was raised (β 1.06 SD, p<0.05, R2 0.37) and its systolic velocity decreased (β −1.05 SD, p<0.05, R2 0.35) in Group 2. With stress, wall motion score index increased (p<0.05) and long axis disturbances worsened only in the same group. Multivariate analysis demonstrated clear relationship between ischaemic LV disturbances, reduced long axis amplitude, global longitudinal systolic strain and early diastolic strain rate. Resting and stress RV lengthening velocity also correlated with CAC score. ConclusionIn symptomatic patients with no obstructive coronary lesions and with more than mild CAC, long axis disturbances and wall motion score index rise occur with stress, at the time of symptom development and correlate with severity of arterial calcification. These findings suggest CAC as a potential mechanism for coronary wall stiffness and consequently exertional ischaemic changes as a result of limited flow reserve.

The effect of right ventricular myocardial remodeling on ventricular function as assessed by two-dimensional speckle tracking echocardiography in patients with tetralogy of Fallot: A single center experience from China

BackgroundPublished correlations between histological abnormalities and right ventricular (RV) function, as evaluated by speckle tracking echocardiography (STE), are scarce in patients with tetralogy of Fallot (TOF). The purpose of the study is to assess age-associated differences in the effect of RV myocardial remodeling on ventricular function in patients with TOF. MethodsOperatively resected crista supraventricularis muscle from 30 patients (median age 12months) undergoing intracardiac repair of TOF were studied by light microscopy. The patients were divided into younger (age at surgery ≤12months) and older (age at surgery >12months) subgroups. The RV global longitudinal peak systolic strain (GLS), strain rate (GLSRs) and early diastolic strain rate (GLSRe) were measured by two-dimensional STE before and 6months after repair. ResultsThe histopathological data revealed hypertrophy of the cardiomyocytes, a thickened endocardium, and increased interstitial and perivascular collagen in RV, which were associated with older age at the time of repair. The RV global systolic and diastolic functions in patients with repaired TOF were increased compared with the preoperative values. The RV cardiomyocyte diameter and collagen volume fraction (CVF) correlated with the preoperative GLS, GLSRs and GLSRe, respectively, in the younger patients (r1=−0.566, P1=0.018; r2=−0.493, P2=0.004; r3=−0.504, P3=0.039). The RV cardiomyocyte diameter and CVF correlated with preoperative GLS, GLSRs and GLSRe, respectively, in the older patients (r1=−737, P1=0.004; r2=−0.588, P2=0.035; r3=−0.812, P3=0.001). The correlation of the RV cardiomyocyte diameter with the postoperative GLS and GLSRe (r1=−665, P1=0.036; r2=−0.787, P2=0.007) and the CVF with the postoperative GLSRs and GLSRe (r1=−762, P1=0.002; r2=−0.713, P2=0.004) were identified only in the older patients. Multivariate analysis indicated that the age at repair was an independent predictor of postoperative GLSRs and GLSRe in all of the patients (β=−0.449, P=0.041; β=−0.607, P=0.004). ConclusionsThe effect of RV myocardial remodeling on preoperative RV function was more pronounced in the older patients with TOF than in the younger ones. Preoperative myocardial remodeling affected the postoperative RV function in the older but not in the younger patients. The age at the time of surgical repair was the independent determinant of the postoperative RV myocardial function.

Age-related changes in familial hypertrophic cardiomyopathy phenotype in transgenic mice and humans.

β-myosin heavy chain mutations are the most frequently identified basis for hypertrophic cardiomyopathy (HCM). A transgenic mouse model (αMHC(403)) has been extensively used to study various mechanistic aspects of HCM. There is general skepticism whether mouse and human disease features are similar. Herein we compare morphologic and functional characteristics, and disease evolution, in a transgenic mouse and a single family with a MHC mutation. Ten male αMHC(403) transgenic mice (at t-5 weeks, -12 weeks, and -24 weeks) and 10 HCM patients from the same family with a β-myosin heavy chain mutation were enrolled. Morphometric, conventional echocardiographic, tissue Doppler and strain analytic characteristics of transgenic mice and HCM patients were assessed. Ten male transgenic mice (αMHC(403)) were examined at ages -5 weeks, -12 weeks, and -24 weeks. In the transgenic mice, aging was associated with a significant increase in septal (0.59±0.06 vs. 0.64±0.05 vs. 0.69±0.11 mm, P<0.01) and anterior wall thickness (0.58±0.1 vs. 0.62±0.07 vs. 0.80±0.16 mm, P<0.001), which was coincident with a significant decrease in circumferential strain (-22%±4% vs. -20%±3% vs. -19%±3%, P=0.03), global longitudinal strain (-19%±3% vs. -17%±2% vs. -16%±3%, P=0.001) and E/A ratio (1.9±0.3 vs. 1.7±0.3 vs. 1.4±0.3, P=0.01). The HCM patients were classified into 1st generation (n=6; mean age 53±6 years), and 2nd generation (n=4; mean age 32±8 years). Septal thickness (2.2±0.9 vs. 1.4±0.1 cm, P<0.05), left atrial (LA) volume (62±16 vs. 41±5 mL, P=0.03), E/A ratio (0.77±0.21 vs. 1.1±0.1, P=0.01), E/e' ratio (25±10 vs. 12±2, P=0.03), global left ventricular (LV) strain (-14%±3% vs. -20%±3%, P=0.01) and global LV early diastolic strain rate (0.76±0.17 s(-1) vs. 1.3±0.2 s-1, P=0.01) were significantly worse in the older generation. In β-myosin heavy chain mutations, transgenic mice and humans have similar progression in morphologic and functional abnormalities. The αMHC(403) transgenic mouse model closely recapitulates human disease.

Ventricular and atrial mechanics and their interaction in patients with congenital scoliosis without clinical heart failure.

This study sought to evaluate left ventricular, right ventricular, and left atrial mechanics and their interactions in patients with congenital scoliosis without clinical heart failure. A total of 23 patients with a median age of 14 years and a median Cobb's angle of 61° were studied. Ventricular and atrial myocardial deformation was measured using speckle tracking echocardiography. The results of the patients were compared with 22 controls. Compared with controls, the patients had a significantly greater annular a velocity (p=0.04) and lower e/a ratio (p=0.03); the left ventricular deformation significantly decreased in radial global (p=0.04) and segmental systolic strain and early diastolic strain rate (p=0.03); the left atrial deformation showed a significantly lower positive strain (p=0.02), greater negative strain (p=0.01), and active contractile strain rate (p=0.01). For the patients, the Cobb's angle was negatively correlated with the left ventricular global radial systolic strain (r=-0.65, p=0.001), left atrial positive strain (r=-0.68, p<0.001), and the left atrial negative strain was positively correlated with the left ventricular circumferential late diastolic strain rate (r=0.46, p=0.01). The left atrial conduit strain rate was positively correlated with the left ventricular circumferential early diastolic strain rate (r=0.42, p=0.03). The left atrial active contractile strain rate was positively correlated with the left ventricular longitudinal late diastolic strain rate (r=-0.4, p=0.03). Impaired left ventricular and altered left atrial mechanics occur relatively early in patients with congenital scoliosis, and are correlated with the severity of their scoliosis. Our findings provide evidence of preclinical heart dysfunction in patients with this disorder.

Right Ventricular Diastolic Performance in Children With Pulmonary Arterial Hypertension Associated With Congenital Heart Disease

Background— Right ventricular diastolic dysfunction influences outcomes in pulmonary arterial hypertension (PAH), but echocardiographic parameters have not been investigated in relation to invasive reference standards in pediatric PAH. We investigated echocardiographic parameters of right ventricular diastolic function in children with PAH in relation to simultaneously measured invasive reference measures. Methods and Results— We prospectively recruited children undergoing a clinically indicated cardiac catheterization for evaluation of PAH and pulmonary vasoreactivity testing. Echocardiography was performed simultaneously with invasive reference measurements by high-fidelity micromanometer catheter. For analysis, patients were divided into shunt and nonshunt groups. Sixteen children were studied. In the group as a whole, significant correlations were found among τ and tricuspid deceleration time, E′ , E/E′ , Time E – E ′ , A wave velocity, and global early and late diastolic strain rate. d p /d t minimum correlated significantly with late diastolic tricuspid annular velocity ( A′ ), tissue Doppler imaging–derived systolic:diastolic duration ratio, and global late diastolic strain rate. End-diastolic pressure correlated significantly with tissue Doppler imaging–derived systolic:diastolic duration ratio. On multivariate analysis, tricuspid deceleration time, Time E – E ′ , and global early diastolic strain rate were independent predictors of τ, whereas tissue Doppler imaging–derived systolic:diastolic duration ratio was an independent predictor of d p /d t minimum. In general, correlations between echocardiographic and invasive parameters were better in the shunt group than in the nonshunt group. Conclusions— Echocardiography correlates with invasive reference measures of right ventricular diastolic function in children with PAH, although it does not differentiate between early versus late diastolic abnormalities. Newer echocardiographic techniques may have added value to assess right ventricular diastolic dysfunction in this population.

The Ratio of Early Mitral Inflow Velocity to Global Diastolic Strain Rate as a Useful Predictor of Cardiac Outcomes in Patients with Atrial Fibrillation

Although the ratio of early mitral inflow velocity (E) to global diastolic strain rate (E'sr) has been correlated with left ventricular filling pressure, its relationship with cardiac outcomes in patients with atrial fibrillation (AF) has never been evaluated. The aim of this study was to examine the ability of E/E'sr ratio in predicting cardiac outcomes in patients with AF. In 190 patients with persistent AF, comprehensive echocardiography was performed, with assessment of E'sr from three standard apical views using the index beat method. Cardiac events were defined as death and hospitalization for heart failure. There were 50 cardiac events, including 22 deaths and 28 hospitalizations for heart failure, during an average follow-up period of 20 months (interquartile range, 14-32 months). Multivariate analysis showed old age, chronic heart failure, and increased E/E'sr ratio (per 10-cm E/E'sr increase; hazard ratio, 1.258; 95% confidence interval, 1.132-1.398, P < .001) were associated with increased cardiac events. In direct comparison, E/E'sr ratio outperformed the ratio of E to early diastolic mitral annular velocity (E') in predicting adverse cardiac events in both univariate and multivariate models (P ≤ .046). Additionally, the addition of E/E'sr ratio to a clinical model including age, chronic heart failure, diabetes, hypertension, left atrial volume index, left ventricular ejection fraction, and E/E' still provided an extra benefit in the prediction of adverse cardiac events (P = .010). E/E'sr ratio is a useful parameter and is stronger than E/E' ratio in predicting adverse cardiac events, and it may offer additional prognostic benefit over conventional clinical and echocardiographic parameters in patients with AF.