Abstract: The aim of the present study was to analyse the adverse effects of SonoVue echo contrast in a consecutive series of 352 cardiac patients during a 4-year period. During 352 consecutive cardiac SonoVue studies, seven patients (2.0%) experienced adverse effects. Four patients (1.1%) had mild allergic reactions causing skin erythema and mild sinus tachycardia, and three patients (0.9%) experienced a severe allergic reaction resulting in (nonfatal) shock. The reported incidence of adverse effects of SonoVue echo contrast in this consecutive series of cardiac patients seems markedly higher than those reported in a company postmarketing analysis.

Abstract: PRINCIPLES: MELAS, or mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes is a new distinctive clinical entity. The current study was designed to assess ascending aortic elasticity in adult patients with MELAS syndrome and in gene carriers, and to compare the results with age- and gender-matched healthy controls. METHODS: The study comprised eight patients with MELAS syndrome and four asymptomatic gene carriers. All subjects underwent complete 2-dimensional transthoracic echocardiography, and systolic and diastolic ascending aortic diameters (SD and DD respectively) were recorded in M-mode 3 cm above the aortic valve from a parasternal long-axis view. Aortic elastic properties were calculated using aortic data and forearm blood pressure values. RESULTS: SD and DD of MELAS patients and gene carriers were enlarged compared with controls. Aortic stiffness index was increased (16.4+/-3.7 vs 3.6+/-1.1, p=0.00001), while aortic strain (0.035+/-0.012% vs 0.146+/-0.050%, p=0.00002) and aortic distensibility (1.03+/-0.30 cm2/dynes 10(-6) vs 4.70+/-1.69 cm2/dynes 10(-6), p=0.0002) were decreased in MELAS patients compared with controls. Aortic elastic properties of gene carriers were between MELAS patients and controls. CONCLUSIONS: Increased ascending aortic stiffness and enlarged aortic dimensions suggesting vascular remodelling were found in MELAS patients as compared with controls.

Abstract: OBJECTIVES: To test the hypothesis that carriers of Dutch founder mutations in cardiac myosin-binding protein C (MYBPC3), without left ventricular hypertrophy (LVH) or electrocardiographic abnormalities, have diastolic dysfunction on tissue Doppler imaging (TDI), which can be used for the screening of family members in the hypertrophic cardiomyopathy (HCM) population. BACKGROUND: TDI is a more sensitive technique for the assessment of left ventricular contraction and relaxation abnormalities than is conventional echocardiography. METHODS: Echocardiographic studies including TDI were performed in genotyped hypertrophic cardiomyopathy patients (genotype-positive, G+/LVH+; n = 27), mutation carriers without LVH (G+/LVH-; n = 27), and healthy controls (n = 55). The identified mutations in MYBPC3 in the G+/LVH+ subjects were c.2864_2865delCT (12 subjects), c.2373dupG (n = 8), and p. Arg943X (n = 7). In the G+/LVH- subjects, the following mutations were identified: c.2864_2865delCT (n = 11), c.2373dupG (n = 8), and p. Arg943X (n = 8). RESULTS: Mean TDI-derived systolic and early and late diastolic mitral annular velocities were significantly lower in the G+/LVH+ subjects compared with the other groups. However, there was no difference between controls and G+/LVH- subjects. Mean TDI-derived late mitral annular diastolic velocities were significantly higher in the G+/LVH- subjects compared with controls and G+/LVH+ subjects. Using a cut-off value of mean +/- 2 SD, an abnormal late mitral annular diastolic velocity was found in 14 (51%) of G+/LVH- patients. There was no difference among the 3 different mutations. CONCLUSIONS: In contrast to earlier reports, mean mitral annular systolic velocity and early mitral annular diastolic velocity velocities were not reduced in G+/LVH- subjects, and TDI velocities were not sufficiently sensitive for determination of the affected status of an individual subject. Our findings, however, support the theory that diastolic dysfunction is a primary component of pre-clinical HCM.

Abstract: OBJECTIVE: To assess real-time 3-dimensional echocardiography (RT3DE)-derived left ventricular (LV) systolic dyssynchrony parameters: (1) normal values, (2) characteristics in patients with heart failure (HF) and a wide or narrow QRS complex, (3) interobserver and intraobserver variability with current state of the art RT3DE hardware and software technology, and (4) incremental value in patients with HF who receive cardiac resynchronization therapy (CRT). METHODS: The study involved 84 patients with HF (mean age 54 +/- 15 years, 50 men) and 60 healthy volunteers (mean age 41 +/- 15 years, 36 men). Semiautomated LV endocardial border tracking was used to calculate regional time-to-minimum systolic volume and to generate parametric maps and the systolic dyssynchrony index (SDI), defined as the standard deviation of time-to-minimum systolic volume of the 16 LV segments expressed in percentage of R-R duration. RESULTS: The volume rate of the RT3DE datasets in patients with HF was 31 +/- 9 Hz (range 15-42 Hz). The normal value of the SDI was 4.1% +/- 2.2% (range <1.0%-8.9%). Patients with HF had a larger SDI (13.4% +/- 8.1%, P < .001). There was only a weak correlation (r2 = 0.07, P < .05) between the QRS duration and the SDI. Interobserver interclass correlation and variability of the SDI depended on image quality (good: 0.993 and 9%, moderate: 0.907 and 16%, respectively). Interobserver agreement for the identification of the most delayed LV segment depended on image quality (good: 90%, moderate: 76%). Thirty-nine patients underwent CRT. At the 12-month follow-up, LV volumetric responders had a significant reduction in the SDI (16.3% +/- 3.3% to 7.7% +/- 2.4%, P < .001). CONCLUSION: With state of the art technology, RT3DE allows reproducible assessment of LV systolic dyssynchrony, which may be useful to identify potential responders to CRT.

Abstract: Non-compaction of the ventricular myocardium is a recently recognized rare disorder of the endomyocardial morphogenesis. The disease can be characterized by systolic and diastolic heart failure, ventricular arrhythmias and systemic embolization. The present case suggests the clinical role of real-time three-dimensional echocardiography in the spatial evaluation of both ventricles in suspected biventricular non-compaction cardiomyopathy.

Abstract: The traditional intraoperative two-dimensional transesophageal echocardiography (2DTEE) has limitations in measuring left ventricular ejection fraction (LVEF) because measurements rely on geometric assumptions. The availability of online software and real time three-dimensional transesophageal echocardiography (RT3D-TEE) makes intraoperative LVEF measurements fast and easy. This is the first report of intraoperative measurement of LVEF and aortic valve area (AVA) by RT3-DTEE in a patient who received transcatheter-based transapical aortic valve implantation.

Abstract: BACKGROUND: Dobutamine stress echocardiography (DSE) suffers from high interobserver and inter-institution variability in the diagnosis of myocardial ischemia. Therefore, we developed a three-dimensional (3D) analysis tool that makes it possible to anatomically align 3D rest and stress data systematically, to generate optimal, nonforeshortened standard anatomical cross sections and to analyse the images synchronized and side-by-side. Aim of the study: To investigate whether this 3D analysis tool could improve interobserver agreement on myocardial ischemia during 3D DSE. METHODS: The study comprised 34 consecutive patients with stable chest pain who underwent both noncontrast and contrast 3D DSE. Two observers scored segmental wall motion using a conventional analysis and the novel analysis with the new 3D tool. RESULTS: The two observers agreed on the presence or absence of myocardial ischemia in 81 of 102 coronary territories (agreement 79%, kappa (kappa) 0.28) during noncontrast 3D imaging and 92 of 102 coronary territories (agreement 90%, kappa 0.65) during contrast-enhanced 3D imaging. With the new 3D analysis software these numbers improved to 98 of 102 coronary territories (agreement 96%, kappa 0.69) during noncontrast 3D imaging and 98 of 102 coronary territories (agreement 96%, kappa 0.82) during contrast-enhanced 3D imaging. CONCLUSION: The use of a 3D DSE analysis tool improves interobserver agreement for myocardial ischemia both for noncontrast and contrast images.

Abstract: Background Noncompaction cardiomyopathy (NCCM) is a recently recognized disorder frequently associated with systolic and diastolic heart failures. This study was designed to examine aortic stiffness in NCCM patients and to compare these results to age- and gender-matched controls. Methods A total of 20 patients with typical echocardiographic features of NCCM (age 38 +/- 16 years, eight males) were investigated. Their results were compared to 20 age- and gender-matched controls. All subjects underwent a complete two-dimensional transthoracic echocardiographic examination. Systolic (SD) and diastolic (DD) ascending aortic diameters were recorded in M-mode at a level of 3 cm above the aortic valve from a parasternal long-axis view. Aortic stiffness index (beta) was calculated as a characteristic of aortic elasticity, as ln(SBP/DBP)/[(SD - DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, respectively, and ln is the natural logarithm. Results The number of noncompacted segments in the NCCM patients was 4.6 +/- 2.0. NCCM patients had significantly increased left ventricular dimensions and reduced left ventricular ejection fraction. Compared to controls, aortic stiffness index (beta) was significantly increased in NCCM patients (8.3 +/- 5.2 vs. 3.5 +/- 1.1, p < 0.001). Conclusion Increased aortic stiffness can be observed in patients with NCCM with moderate to severe heart failure. These alterations may be due to neurohormonal changes in heart failure.

Abstract: PURPOSE. Leber hereditary optic neuropathy (LHON) is recognized as the most common cause of isolated blindness in young men. The current study was designed to test whether LHON as a mitochondrial disease is associated with vascular functional alterations characterized by aortic elastic properties during echocardiography. METHODS. A total of 19 patients with typical features of LHON aged 42+/-13 years (10 males) were included. Their results were compared to 19 age- and gender-matched healthy controls. Aortic stiffness index was calculated from the echocardiographically derived aortic diameters and the clinical blood pressure data. RESULTS. In this patient population, the point mutation was present in 3460G>A position in five cases, in 11778G>A position in five cases, and in 14484T>C position in nine patients. Diastolic aortic diameter (26.0+/-2.5 mm vs 28.4+/-4.1 mm, p< 0.05) and aortic stiffness index (5.1+/-2.6 vs 12.0+/-7.9, p< 0.05) were significantly increased in LHON patients compared to controls. CONCLUSIONS. Aortic stiffness can be increased in LHON disease, but further studies are warranted to confirm these findings in a larger LHON patient population with a more reliable method focusing on the pathophysiologic background.

Abstract: Background and aim of the study: Non-compaction cardiomyopathy (NCCM) is a disorder characterized by loosened, spongy myocardium associated with a high incidence of heart failure and systemic embolization. The mitral annulus (MA) is an important component of the mitral valve apparatus, and plays a role in left ventricular and left atrial function. The study aim was to use real-time three-dimensional echocardiography (RT3DE) in the assessment of MA size and function in patients with NCCM. Methods: The study comprised the following patient populations: 20 patients with an established diagnosis of NCCM; 20 with an established diagnosis of dilated cardiomyopathy (DCM); and 16 control subjects. RT3DE was used to measure MA annulus diameter, MA fractional area change (MAFAC(3D)) and MA fractional shortening (MAFS(3D)). Results: The left ventricular ejection fraction (LVEF) in NCCM patients (38.0 +/- 18.3%) was higher than in DCM patients (18.0 +/- 4.1%), but lower than in controls (56.9 +/- 9.2%) (p <0.05). The prevalence and severity of mitral regurgitation were comparable in the NCCM and DCM groups, but higher than in controls (p <0.05). The MA area was significantly larger, while MAFAC(3D) and MAFS(3D) were significantly impaired in NCCM patients compared to controls. Both, in systole and diastole, the MA diameter and area were larger in DCM than NCCM patients, but no difference was observed in MAFAC(3D) and MAFS(3D). The number of non-compacted segments did not correlate with MA diameter and area, MAFAC(3D) and MAFS(3D). MAFAC(3D) was significantly correlated with LVEF in NCCM (r = 0.43, p <0.05) and in DCM (r = 0.47, p <0.05). Conclusion: Mitral annulus enlargement and functional impairment were both present in NCCM patients, with a higher incidence and severity of mitral regurgitation.

Abstract: Methods The study included 25 hypertrophic cardiomyopathy (HCM) patients (15 non-obstructive and 10 obstructive) and 25 controls for assessment of left atrial (LA) volume, mass and function by two-dimensional echocardiography. Measurement included mean LA diameter (LAD), LA mass = {(mean LAD + anterior LA wall + posterior LA wall)(3) - mean LAD(3)} x 0.8 + 0.6, LA volume = [(8/3 pi L center dot A1 center dot A2), where L is LA length, A1 and A2 are LA area in 4-chambers and 2-chambers, respectively] including maximum (V-max), minimum (V-min), and pre-atrial contraction (Vpre-A), total atrial stroke volume (TA-SV), TA emptying fraction (TA-EF), active atrial SV (AA-SV), AA-EF, passive atrial SV (PA-SV), PA-EF, atrial expansion index (AEI), and LA kinetic energy (LA-KE) = 1/2 x AA-SV x P x V-2. Results LAD, LA mass, V-max, V-min, and Vpre-A were significantly higher in HCM than controls. TA-SV and TA-EF were comparable in both HCM subgroups and controls. AA-SV and LA-KE were significantly higher in both HCM subgroups than controls. LA-KE was significantly higher in obstructive HCM than non-obstructive (P < 0.001). PA-EF and AEI were significantly lower in obstructive HCM than controls (P < 0.05). Conclusion HCM is associated with increased LA size and augmented LA pump function especially obstructive type. LA conduit and reservoir functions are impaired in obstructive HCM.

Abstract: Mucopolysaccharidosis type I (MPS IS) is a rare autosomal recessive disease caused by a deficiency of the lysosomal enzyme alpha-L-iduronidase, which is involved in the degradation of sulfated glycosaminoglycans (GAGs). The deficiency results in the intra-and pericellular accumulation of the GAGs heparan sulfate and dermatan sulfate. Eight adult patients with typical features of MPS IS aged 31.5 +/- 6.8 years (five men) were included and compared to age-and gender-matched controls. With transthoracic echocardiography, cyclic ascending aortic diameter changes were measured and ascending aortic elastic properties were calculated to characterize aortic elasticity. In MPS IS patients, aortic stiffness index was significantly increased (23.1 +/- 10.4 vs 3.9 +/- 1.5, P < 0.001), while aortic distensibility was significantly decreased (1.6 +/- 0.8 vs 1.6 +/- 1.9 Ca-2/dynes 10(-6), P < 0.001) compared to age-and sex-matched controls. The results of the present study demonstrate that in addition to the known cardiac complications, MPS IS patients have an impairment of ascending aortic elasticity. Further follow-up studies are needed to examine arterial elasticity using other methods in this patient population, and to detect possible effects of enzyme replacement therapy.

Abstract: The present study evaluated LV volumes, ejection fraction (LVEF) and stroke volume (SV) obtained by 64-MDCT and to compare these data with those obtained by second harmonic 2D Echo, in patients referred for non-invasive coronary vessels evaluation. The most common technique in daily clinical practice used for determination of LV function is two-dimensional echocardiography (2D-TTE). Multi-detector computed tomography (MDCT) is an emerging new technique to detect coronary artery disease (CAD) and was recently proposed to assess LV function. 93 patients underwent to 64-MDCT for LV function and volumes assessment by segmental reconstruction algorithm (Argus) and compared with recent (2 months) 2D-TTE, all images were processed and interpreted by two observers blinded to the Echo and MDCT results. A close correlation between TTE and 64 MDCT was demonstrated for the ejection fraction LVEF (r=0.84), end-diastolic volume LVEDV (r=0.80) and end-systolic volume LVESV (r=0.85); acceptable correlation was recruited for stroke volume LVSV (r=0.58). Optimal results were recruited for inter-observer variability for 64-MDCT measured in 45 patients: LVESV (r=0.82, p<0.001), LVEDV (r=0.83, p<0.001), LVEF (r=0.69, p<0.002) and SV (r=0.66, p<0.001). Our results, showed that functional and temporal information contained in a coronary 64-MDCT study can be used to assess left ventricular (LV) systolic function and LV dimensions with good reproducibility and acceptable correlation respect to 2D-TTE. The combination of non-invasive coronary artery imaging and assessment of global LV function might became in the future a fast and conclusive cardiac work-up in patients with CAD.

Abstract: AIMS: Although safety and feasibility studies have been published, there are few reports dedicated to the echocardiographic evaluation of patients following percutaneous aortic valve replacement (PAVR). This report describes the early echocardiographic evaluation of patients undergoing PAVR with the CoreValve Revalving System. METHODS AND RESULTS: The population consisted of 33 consecutive patients with aortic stenosis who underwent successful PAVR. Echocardiograms were performed pre-treatment (123+/-110 days prior), post-treatment (6+/-2 days) and post-discharge (80+/-64 days). Aortic valve function and left ventricular dimensions, systolic and diastolic function were assessed pre- and post-implantation. The mean age was 81+/-7 years and the mean Logistic Euroscore was 20+/-12. Following PAVR, the mean transaortic valve gradient decreased (46+/-16 mmHg pre-treatment vs. 12+/-7 mmHg post-treatment vs. 9+/-5 mmHg post-discharge, p<0.001) and the mean effective orifice area increased (0.75+/-0.23 cm2 pre-treatment vs. 1.97+/-0.85 cm2 post-treatment vs. 1.72+/-0.45 cm2 post-discharge, p<0.001). There was no significant change in mean ejection fraction (41+/-12% pre-treatment vs. 46+/-15% post-treatment vs. 44+/-13% post-discharge, p=0.44). Approximately two-thirds of patients had no change in diastolic function at follow-up. CONCLUSION: Following implantation, there was a sustained decrease in aortic valve gradient and increase in aortic valve area. In addition, the mean ejection fraction did not change significantly and in the majority of patients, diastolic function was unchanged.

Abstract: Background: Determination of left atrial (LA) size and function is important in clinical decision-making. Calculation of LA volume (LAV) is the most accurate index of LA size. Aim: To compare real-time 3-dimensional echocardiography (RT3DE) and 2-dimensional echocardiography (2DE) for calculation of LAV and function. Methods: Fifty patients were studied using 2DE and RT3DE for calculating LAV including: Maximum (V max), minimum (V min) and preatrial contraction (V pre A) volumes. For 2DE, the formula: LAV = 8(A1) (A2)/ 3 pi (L) was used, while for RT3DE, offline analysis was performed using commercially available software. LA function indices including Total Atrial Stroke Volume (TASV), active ASV (AASV), Total Atrial Emptying Fraction (TAEF), active AEF (AAEF), passive AEF (PAEF), and Atrial Expansion Index (AEI) were calculated. Results: Patients were classified into 2 equal groups: group I with normal V max (<50 ml) and group II with V max (= 50 ml). Good correlation was obtained between RT3DE and 2DE for LAV (r = 0.64, p = 0.001) in group I and (r = 0.83, p<0.0001) in group II. In group I, LAV and functions showed no significant difference by both techniques, while in group II, the V min and V pre A were significantly lower by RT3DE than 2DE (p= 0.009, 0.006). TAEF, AEI, and PAEF indices were significantly higher by RT3DE than 2DE in group II. Conclusion: RT3DE provides a reproducible assessment of active and passive LA function by volumetric cyclic changes. It is comparable and may be superior to 2DE due to its higher sensitivity to volume changes. (c) 2007 Published by Elsevier Ireland Ltd.

Abstract: Background: Description and quantification of regional left ventricular (LV) rotation and the time course of LV rotation might provide further insight into LV function. Methods: The study comprised 60 healthy volunteers (age 39 +/- 15 years, 31 men) in whom complete global and regional LV rotation could be assessed at both the basal and apical LV level with speckle tracking echocardiography, using QLAB advanced quantification software version 6.0 (Philips, Best, The Netherlands). Results: At the LV basal level, a brief counterclockwise rotation from aortic valve opening until 25% ejection was seen in the anterior segments (anterior, anteroseptal, anterolateral) only. Clockwise rotation in the anterior segments at the basal level was decreased as compared to the posterior segments (inferior, inferoseptal, inferolateral) from 25% ejection until aortic valve closure. At the LV apical level, all segments showed a brief clockwise rotation during the isovolumic contraction phase. Also, at this level there were no differences in regional LV rotation at any other moment during the cardiac cycle. There was a marked de-rotation from the moment of maximal rotation until E-peak at the LV basal level (79 +/- 18%) whereas de-rotation during this interval was less pronounced at the LV apical level (55 +/- 21%). Only at the LV basal level significant linear relationships were seen between the E/A ratio and de-rotation extent and velocity from mitral valve opening until E-peak (R(2)= 0.42 and R(2)= 0.40, respectively, both P < 0.001). Conclusion: In the normal human heart significant regional differences in LV rotation and de-rotation exist.

Abstract: Patients with heart failure and mechanical dyssynchrony suffer a progressive increase in left ventricular (LV) mass and asymmetrical regional hypertrophy with eventual poor prognosis. The present study sought to investigate whether cardiac resynchronization therapy (CRT) could reverse these abnormalities. The study included 66 consecutive heart failure patients who received CRT. All patients underwent serial evaluation before, 3 months after, and 12 months after CRT. At 12 months after CRT, 50 patients (76%) were echocardiographic volumetric responders, defined as a > 15% reduction in LV end-systolic volume. LV end-systolic volume was decreased from 214 +/- 97 ml to 179 +/- 88 ml at 3 months and was further decreased to 158 +/- 86 ml at 12 months after CRT (all p < 0.01). LV ejection fraction was improved from 18% +/- 4% to 28% +/- 7% (p < 0.001) at 3 months without further change at 12 months after CRT. LV mass was reduced from 242 +/- 52 g to 222 +/- 45 g at 3 months and was further reduced to 206 +/- 50 g at 12 months after CRT (all p < 0.01). Improvement of LV geometry was seen as improvements of the end-diastolic (1.64 +/- 0.14 vs 1.77 +/- 0.17, p < 0.001) and the end-systolic (1.63 +/- 0.14 vs 1.90 +/- 0.22, p < 0.001) sphericity indexes, respectively, at 3 months, without further significant, changes at 12 months after CRT. Volumetric responders had a reduction in LV mass from 240 +/- 50 to 210 +/- 38 at 3 months, and LV mass was further reduced to 186 +/- 37 g at 12 months after CRT (all p < 0.01). In contrast, nonresponders had a progressive increase in LV mass from 248 +/- 59 g to 258 +/- 54 g at 3 months, and LV mass was further increased to 269 +/- 60 g at 12 months after CRT (all p < 0.05). Likewise, only in volumetric responders, regression of the asymmetric hypertrophy of the lateral wall was noted. In conclusion, CRT results in not only volumetric improvement but also in true reverse LV structural remodeling, evidenced by progressive reduction in LV mass and restoration of regional wall symmetry.

Abstract: Background Noncompaction cardiomyopathy (NCCM) is a rare disorder with persistance of the embryonic pattern of myoarchitecture. NCCM is characterized by loosened, spongy myocardium associated with a high incidence of systolic and diastolic left ventricular (LV) dysfunction and heart failure (HF). It is known that LV dysfunction contributes to elevated left atrial (LA) and pulmonary vascular pressures, however atrial function has not been examined in NCCM. The objective of the present study was to assess LA systolic function characterized by LA ejection force (LAEF) in NCCM patients using real-time three-dimensional echocardiography (RT3DE) and to compare to control subjects. Methods The study comprised 17 patients with an established diagnosis of NCCM and their results were compared to 17 healthy age-matched controls with no evidence of cardiovascular disease. Forty-one percent of NCCM patients were in NYHA functional class II / III HF. Previously proposed echocardiographic diagnostic criteria for NCCM were used. All patients underwent conventional two-dimensional echocardiography and RT3DE. LAEF was measured based on MA annulus diameter (LAEF(3D-MAD)) and area (LAEF(3D-MAA)) using RT3DE. Results The presence and severity of mitral regurgitation were more frequent in NCCM patients than in control subjects. LV diameters and mitral annulus were significantly increased in NCCM patients. Compared with control subjects, both LAEF(3D-MAD) (3.8 +/- 2.2 vs 2.3 +/- 1.0 kdyne, P < 0.05) and LAEF(3D-MAA) (12.7 +/- 7.6 vs 4.9 +/- 2.1 kdyne, P < 0.01) were significantly increased in NCCM patients. Conclusions LAEF as a characteristic of LA systolic function is increased in NCCM patients compared to normal individuals. These results can suggest compensating left atrial work against the dysfunctional LV in NCCM patients.

Abstract: Objectives: To assess mechanisms of myocardial perfusion impairment in patients with hypertrophic cardiomyopathy (HCM). Methods: Fourteen patients with obstructive HCM ( mean (SD) age 53 ( 10) years, 11 men) underwent intravenous adenosine myocardial contrast echocardiography (MCE), positron emission tomography ( PET) and cardiac catheterisation. Fourteen healthy volunteers ( mean age 31 ( 4) years, 11 men) served as controls. Relative myocardial blood volume (rBV), exchange flow velocity (beta), myocardial blood flow (MBF), MBF reserve (MFR) and endocardial-to-subepicardial (endo-to-epi) MBF ratio were measured from the steady state and contrast replenishment time-intensity curves. Results: Patients with HCM had lower rest MBF ( for LVRPP-corrected)-mean ( SD) (0.92 (0.12) vs 1.13 (0.25) ml/min/g, p, 0.01)-and hyperaemic MBF-(2.56 (0.49) vs 4.34 (0.78) ml/min/g, p, 0.01) than controls. Resting rBV was lower in patients with HCM (0.094 (0.016) vs 0.138 (0.014) ml/ml), and during hyperaemia (0.104 (0.018) ml/ml vs 0.185 (0.024) ml/ml) ( all p < 0.001) than in controls. b tended to be higher in HCM at rest (9.4 (4.6) vs 7.7 (4.2) ml/min) and during hyperaemia (25.8 (6.4) vs 23.1 (6.2) ml/min) than in controls. Septal endo-to-epi MBF decreased during hyperaemia ( 0.86 (0.15) to 0.64 (0.18), p < 0.01). rBV was inversely correlated with left ventricular (LV) mass index ( p < 0.05). Both hyperaemic and endo-to-epi MBF were inversely correlated with LV end-diastolic pressure, LV mass index, and LV outflow tract pressure gradient ( all p < 0.05). MCE-derived MBF correlated well with PET at rest (r = 0.84) and hyperaemia ( r = 0.87) ( all p < 0.001). Conclusions: In patients with HCM, LV end-diastolic pressure, LV outflow tract pressure gradient, and LV mass index are independent predictors of rBV and hyperaemic MBF.

Abstract: AIM: To detect the accuracy of real-time three-dimensional echocardiography (RT3DE) and two-dimensional echocardiography (2DE) for tricuspid annulus (TA) assessment compared with magnetic resonance imaging (MRI). METHODS: Thirty patients (mean age 34 +/- 13 years, 60% males) in sinus rhythm were examined by MRI, RT3DE, and 2DE for TA assessment. End-diastolic and end-systolic TA diameter (TAD) and TA fractional shortening (TAFS) were measured by RT3DE, 2DE, and MRI. End-diastolic and end-systolic TA area (TAA) and TA fractional area changes (TAFAC) were measured by RT3DE and MRI. End-diastolic and end-systolic right ventricular (RV) volumes and ejection fraction (RV-EF) were measured by MRI. RESULTS: The TA was clearly delineated in all patients and visualized as an oval-shaped by RT3DE and MRI. There was a good correlation between TAD(MRI) and TAD(3D) (r = 0.75, P = 0.001), while TAD(2D) was fairly correlated with TAD(3D) and TAD(MRI) (r = 0.5, P = 0.01 for both). There were no significant differences between RT3DE and MRI in TAD, TAA, TAFS, and TAFAC measurements, while TAD(2D) and TAFS(2D) were significantly underestimated (P < 0.001). TAFS(2D) was not correlated with RV-EF, while TAFS(3D) and TAFAC(3D) were fairly correlated with RV-EF (r = 0.49, P = 0.01, and r = 0.47, P = 0.02 respectively). CONCLUSION: RT3DE helps in accurate assessment of TA comparable to MRI and may have an important implication in the TV surgical decision-making processes. RT3DE analysis of TA function could be used as a marker of RV function.

Abstract: Objective. We evaluated two different commercially available real-time 3-dimensional echocardiographic semiautomated border detection algorithms for left ventricular (LV) volume analysis in patients with cardiomyopathy and distorted IV geometry. Methods: A total of 53 patients in sinus rhythm with various types of cardiomyopathy (mean age 56 +/- 11 years, 28 men) and adequate 2-dimensional image quality were included. The real-time 3-dimensional echocardiographic multiplane interpolation (MI) and full volume reconstruction (FVR) methods were used for IV volume analysis. Magnetic resonance imaging was used as the reference method. Results: A strong correlation (R-2 > 0.95) was found for all IV volume and ejection fraction measurements by either real-time 3-dimensional echocardiographic method. Analysis time was shorter with the FVR method (6 +/- 2 vs 15 +/- 4 minutes, P <.01) as compared with the MI method. Bland-Altman analysis showed greater underestimation of end-diastolic and end-systolic volumes by MI compared with FVR. For the MI method a bias of -24.0 mL (-15.0% of the mean) for end-diastolic volume and -11.3 mL (-18.0% of the mean) for end-systolic volume was found. For FVR analysis these values were -9.9 mL (-6.0% of the mean) and -5.0 mL (-9.0% of the mean), respectively. Ejection fraction was similar for the MI and FVR method with a mean difference compared with magnetic resonance imaging of 0.6 (1.0%) and 0.8 (1.3%), respectively. Conclusion: In patients with cardiomyopathy, distorted LV geometry, and good 2-dimensional image quality, the FVR method is faster and more accurate than the MI method in assessment of LV volumes.

Abstract: Aims The current study sought to assess if pre-implantation laterat-to-septal delay (LSD) > 60 ms assessed by spectral pulsed-wave myocardial tissue Doppler imaging (PW-TDI) could predict successful long-term outcome after cardiac resynchronization therapy (CRT). Methods and results Sixty patients (72% mates, mean age 59 +/- 10 years) who were referred for CRT according to the ACC/ESC guidelines were enrolled in the study. All patients underwent spectral PW-TDI before and 1 year after CRT. Two left ventricular (I]V) dyssynchrony time intervals, To and Tp (time to onset and peak of I]V myocardial velocity, respectively), LSD were recorded. Left ventricular dyssynchrony was defined as LSD > 60 ms. Clinical response was defined as an improvement in > 1 NYHA class plus improvement in 6-min walk distance (6MWD) >= 25%, echocardiographic response was defined as a >= 15% reduction in LV end-systolic volume (LV-ESV). One year after CRT, 50 patients (83%) were clinical responders and 47 patients (78%) were echocardiographic responders. Both To and Tp LV dyssynchrony indices failed to predict echocardiographic CRT outcome. In addition, there were no significant differences between 'synchronous' and 'dyssynchronous' patient populations at baseline or follow-up in either clinical (NYHA class and 6MWD) or echocardiographic (LV ejection fraction, LV enddiastolic, and end-systolic) variables. Conclusion The great majority of patients referred for CRT benefit clinically from it. However, spectral PW-TDI failed to predict CRT outcome. When PW-TDI dyssynchrony was applied for selection of proper CRT patients, up to 80-86% of the patients with synchronous LSD that had proven clinical and echocardiographic benefit from CRT would have been denied CRT.

Abstract: Background: To evaluate the prognostic value of tissue Doppler imaging (TDI)-derived parameters (E/E' ratio and Tei index) in heart failure (HF) patients who underwent cardiac resynchronization therapy (CRT). Methods and Results: The study comprised 74 consecutive HF patients (mean age 60 +/- 11 years) who underwent CRT. Echocardiography including TDI measurements was performed in all patients at baseline and 3 months after CRT. During a median follow-up period of 720 days (range 210 to 1020 days), 21 patients (28%) had events (8 deaths, and hospitalization for HF in the remaining 13). From the baseline clinical and echocardiography data, univariable Cox-regressions analysis revealed that only diabetes (hazard ratio [HR] 3.703, P <.01), E/A ratio (HR 3.492, P <.001), and E/E' ratio (HR 1.130, P <.001) were predictors for cardiac events. From the 3-month follow-up data, the E/A ratio (HR 2.988, P <.005), E/E' ratio (HR 1.170, P <.001), left ventricular ejection fraction (HR 0.835, P <.01), deceleration time (HR 0.977, P <.05), and the Tei index (HR 15.784, P <.001) were predictors for cardiac events. After multivariable analysis, only diabetes (HR 5.544, P <.05), the 3-month E/E' ratio (HR 1.229, P <.001), and change in Tei index (HR 32.174, P <.001) were independent predictors for cardiac events. Patients with a high baseline and 3-month follow-up E/E' ratio had an 88% cardiac event rate. Conclusions: The Tei index and E/E' ratio are independent predictors of poor response and cardiac events after CRT.

Abstract: We evaluated the value of baseline parameters derived from tissue Doppler imaging (TDI) for event prediction in patients with heart failure (HF) secondary to ischemic and non-ischemic cause who underwent cardiac resynchronization therapy (CRT). Seventy-four consecutive patients with HF (mean age 59 +/- 11 years) underwent CRT. Baseline clinical parameters included New York Heart Association class, 6-minute walking distance, HF cause, and diabetes. TDI-derived parameters included lateral and septal OF ratios defined as peak early left ventricular (LV) filling velocity (E wave) to TDI-derived peak early diastolic velocity of the mitral annulus (E' wave). During a median follow-up of 720 days, 21 patients (28%) had cardiac death or hospitalization for HF. These patients more often had an ischemic cause (p < 0.05), diabetes (p < 0.05), and restrictive filling (p < 0.001), less often had LV dyssynchrony (p < 0.05), and had higher septal and lateral OF ratios (p < 0.001 for the 2 comparisons). In a multivariable model using a forward selection algorithm, only the lateral OF ratio remained an independent predictor of,cardiac outcome. After 3 months of CRT, TDI-derived systolic mitral annular systolic and diastolic velocities improved significantly in nonischemic patients for the septal and lateral sides. In contrast, in ischemic patients no significant improvements were seen. Significant improvements were seen in septal and lateral OF ratios in ischemic and nonischemic patients. However, the improvement in lateral OF ratio was significantly less and absolute 3-months OF ratios were worse in ischemic patients. In conclusion, baseline lateral E/E' ratio is an independent predictor for cardiac events in patients with HF treated with CRT. The worse clinical outcome in ischemic patients may be due to failure of improvement in systolic and diastolic mitral annular velocities after CRT, resulting in a less pronounced improvement in LV filling pressures as demonstrated by this OF ratio. (c) 2007 Elsevier Inc. All rights reserved.

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Abstract: Recently, debate has erupted about the clinical significance of cardiovascular shunts. Several major health problems such as stroke and migraine have been associated with patent foramen ovate (PFO) with right-to-left shunt (RLS). The nature of the relationship between these syndromes and PFO is not clearly understood. Technical advances have led to more therapeutic options including device closure of PFO, hence prevention of such a PFO-related stroke has become feasible. Therefore, optimal diagnosis of PFO has become of greater clinical importance. Contrast echocardiography with non-transpulmonary contrast agents has been the cornerstone in diagnosis of PFO with RLS for over four decades. Despite being a relatively invasive procedure, transesophageal echocardiography (TEE) is considered the gold standard for detection of RLS. Several other echocardiographic techniques such as transthoracic echocardiography (TTE) with second harmonic imaging and transcranial Doppler ultrasonography (TCD) have shown increased sensitivity and specificity compared to TEE for the detection of PFO with RLS. Moreover, improvement of skills and techniques used for detection of these shunts has led to greater detection of small and large sized RLS in the echocardiographic laboratory. This review gives and overview of the echocardiographic techniques, contrast agents and manoeuvres used for detection of the major cardiovascular shunts and their clinical relevance to major health problems. (c) 2007 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.

Abstract: Dobutamine stress echocardiography is an accepted tool for the diagnosis of coronary artery disease. Some investigators have claimed that 3-dimensional imaging improves the diagnostic accuracy of dobutamine stress echocardiography. The purpose of the present investigation was to examine the role of contrast echocardiography in the improvement of segmental quality and interobserver agreement during stress real-time 3-dimensional echocardiography (RT3DE). The study comprised 36 consecutive patients with stable chest pain referred for routine stress testing. Three-dimensional images were acquired with an RT3DE system with an X4 matrix-array transducer. All available reconstructed 2-dimensional segments were graded as optimal, good, moderate, or poor. Wall motion was scored as normal, mild hypokinesia, severe hypokinesia, akinesia, or dyskinesia. At peak stress, 466 of the 612 segments (76%) could be analyzed during conventional RT3DE. With contrast-enhanced RT3DE, the number of available segments increased to 553 (90%). The image quality index during conventional RT3DE was 2.2, whereas with contrast-enhanced RT3DE, it was 3.1. With conventional RT3DE, 2 independent observers agreed on the diagnosis of myocardial ischemia in 85 of 108 coronary territories (79%, kappa = 0.26). With contrast-enhanced RT3DE, agreement increased to 95 of 108 coronary territories (88%, kappa = 0.59). Study agreement on myocardial ischemia was present in 26 of 36 studies (72%, kappa = 0.43) with conventional RT3DE and in 32 of 36 studies (89%, kappa = 0.77) with contrast-enhanced RT3DE. In conclusion, during stress RT3DE, contrast-enhanced imaging significantly decreases the number of poorly visualized myocardial segments and improves interobserver agreement for the diagnosis of myocardial ischemia.

Abstract: Background: Pompe's disease, also known as acid maltase deficiency or glycogen storage disease type II, is an autosomal recessive disorder in which deficient activity of the enzyme acid alpha-glucosidase causes intra-lysosomal accumulation of glycogen in muscle and other tissues. The current study was designed to assess aortic stiffness index (beta), as a characteristic of aortic elasticity during transthoracic echocardiography in patients with Pompe's disease. Methods: A total of 17 patients (age 44 +/- 8 years, 5 males) with Pompe's disease were studied. Their results were compared to 17 age-and gender-matched controls. In all patients, the ascending aorta was recorded with M-mode echocardiography. beta was calculated as ln(SBP/DBP)/[(SD-DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, SD and DD are the systolic and diastolic aortic diameters, and 'In' is the natural logarithm. Results: Diastolic aortic diameter was 27.4 +/- 2.4 mm in Pompe patients and 25.6 +/- 2.7 mm in controls (P < 0.05). Systolic aortic diameters did not differ between the groups (29.4 +/- 2.5 mm vs 28.3 +/- 2.4 mm, P= ns). Aortic stiffness index (beta) was increased in Pompe patients compared to controls (14.6 +/- 10.1 vs 5.1 +/- 2.6, P < 0.001). Conclusions: The results of this study indicate that aortic stiffness is increased in patients with Pompe's disease. This may be due to glycogen storage in the vessel wall causing reduced vascular elasticity. (C) 2006 Elsevier Ireland Ltd. All rights reserved.

Abstract: Aims: The aim of the present study was to assess the relative prognostic value of coronary flow reserve (CFR) and diabetes mellitus (DM) in patients with suspected coronary artery disease (CAD). Methods: We prospectively studied 347 inbospital patients with chest pain. Coronary angiography was performed in 281 patients (81%). All patients underwent a transthoracic echocardiographic study to evaluate left ventricular function and a stress vasodilator transoesophageal echocardiographic study to evaluate simultaneously CFR and the degree of aortic atherosclerosis (AA). The primary outcome of the study was cardiovascular mortality. Results: During a mean follow-up of 41 12 months, 22 patients suffered cardiovascular death. Diabetic patients had a significantly higher AA grade and tended to have a lower CFR and more often significant CAD. Patients with normal CFR had less often significant CAD and tended to have less often DM. Significant univariable predictors of cardiovascular survival were DM, LV end-diastolic diameter, CFR and AA grade. Multivariable regression analysis showed that only CFR (hazard ratio (HR) 2.9, P = 0.01) and diabetes (HR 3.1, P = 0.01) were independent predictors of cardiovascular survival. Conclusions: CFR and DM evaluations offer complementary information during vasodilator stress TEE testing. Patients with reduced CFR (impaired microcirculatory function) and DM have the worst prognosis. (C) 2007 Elsevier Ireland Ltd. All rights reserved.

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Abstract: Background: Cardiac involvement in mucopolysaccharidosis type I (MPS I) has been studied primarily in its most severe forms. Cardiac involvement, particularly left ventricular (LV) systolic and diastolic function, in the attenuated form of MPS I is less well known. Methods: Cardiac function was prospectively investigated in 9 adult patients with the attenuated form of MPS I. All patients underwent 12-lead electrocardiography, 24 h Holter monitoring and two-dimensional echocardiography including tissue Doppler imaging (TDI). Eighteen age- and sex-matched healthy volunteers served as a control group. Results: Aortic, mitral and tricuspid valve thickening was seen in, respectively, 5 (56%), 4 (44%) and 2 (22%) patients. Moderate mitral valve stenosis was seen in 1 patient and moderate aortic stenosis in 2 patients. All patients had mild-to-moderate aortic and mitral valve regurgitation and 6 patients (67%) had mild-to-moderate tricuspid valve regurgitation. Despite normal LV dimensions, ejection fraction and mass index, MPS patients had lower mean systolic mitral annular velocities (6.1 +/- 0.6 vs 9.1 +/- 1.4 cm/s, p < 0.01) compared to normal control subjects. Similarly, mean early diastolic mitral annular velocities were lower in MPS patients (7.8 +/- 0.9 vs 13.3 +/- 3.3 cm/s, p < 0.01). Conclusion: MPS I patients with the attenuated phenotype have not only valvular abnormalities but also LV diastolic and systolic abnormalities.

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Abstract: BACKGROUND: Determination of left atrial (LA) size and function is important in clinical decision-making. Calculation of LA volume (LAV) is the most accurate index of LA size. AIM: To compare real-time 3-dimensional echocardiography (RT3DE) and 2-dimensional echocardiography (2DE) for calculation of LAV and function. METHODS: Fifty patients were studied using 2DE and RT3DE for calculating LAV including: Maximum (V max), minimum (V min) and pre-atrial contraction (V pre A) volumes. For 2DE, the formula: LAV=8(A1) (A2)/3pi (L) was used, while for RT3DE, offline analysis was performed using commercially available software. LA function indices including Total Atrial Stroke Volume (TASV), active ASV (AASV), Total Atrial Emptying Fraction (TAEF), active AEF (AAEF), passive AEF (PAEF), and Atrial Expansion Index (AEI) were calculated. RESULTS: Patients were classified into 2 equal groups: group I with normal V max (<50 ml) and group II with V max (>/=50 ml). Good correlation was obtained between RT3DE and 2DE for LAV (r=0.64, p=0.001) in group I and (r=0.83, p<0.0001) in group II. In group I, LAV and functions showed no significant difference by both techniques, while in group II, the V min and V pre A were significantly lower by RT3DE than 2DE (p=0.009, 0.006). TAEF, AEI, and PAEF indices were significantly higher by RT3DE than 2DE in group II. CONCLUSION: RT3DE provides a reproducible assessment of active and passive LA function by volumetric cyclic changes. It is comparable and may be superior to 2DE due to its higher sensitivity to volume changes.

Abstract: Recently, debate has erupted about the clinical significance of cardiovascular shunts. Several major health problems such as stroke and migraine have been associated with patent foramen ovale (PFO) with right-to-left shunt (RLS). The nature of the relationship between these syndromes and PFO is not clearly understood. Technical advances have led to more therapeutic options including device closure of PFO, hence prevention of such a PFO-related stroke has become feasible. Therefore, optimal diagnosis of PFO has become of greater clinical importance. Contrast echocardiography with non-transpulmonary contrast agents has been the cornerstone in diagnosis of PFO with RLS for over four decades. Despite being a relatively invasive procedure, transesophageal echocardiography (TEE) is considered the gold standard for detection of RLS. Several other echocardiographic techniques such as transthoracic echocardiography (TTE) with second harmonic imaging and transcranial Doppler ultrasonography (TCD) have shown increased sensitivity and specificity compared to TEE for the detection of PFO with RLS. Moreover, improvement of skills and techniques used for detection of these shunts has led to greater detection of small and large sized RLS in the echocardiographic laboratory. This review gives and overview of the echocardiographic techniques, contrast agents and manoeuvres used for detection of the major cardiovascular shunts and their clinical relevance to major health problems.

Abstract: OBJECTIVES: We sought to assess the intra- and extra-vascular mechanisms of myocardial perfusion impairment in patients with hypertrophic cardiomyopathy (HCM) by real-time myocardial contrast echocardiography (MCE) and positron emission tomography (PET). METHODS: Fourteen patients with obstructive HCM (mean age 53 +/- 10 years, 11 men) underwent intravenous adenosine MCE, PET and cardiac catheterization. Fourteen healthy volunteers (mean age 31 +/- 4 years, 11 men) served as a control group. The relative myocardial blood volume (rBV), its exchange flow velocity (beta), myocardial blood flow (MBF), its reserve (MFR) and the endocardial-to-subepicardial (endo-to-epi) MBF ratio were measured from the steady state and replenishment time-intensity curves of the ultrasound contrast agents. RESULTS: HCM patients had lower values of rest MBF (for LVRPP-corrected) with MCE (0.92 +/- 0.12 vs. 1.13 +/- 0.25 ml.min-1.g-1, P <0.01) compared to healthy controls. Hyperaemic MBF were also lower (2.56 +/- 0.49 vs. 4.34 +/- 0.78 ml.min-1.g-1, P <0.01) resulting in lower MFR (2.80 +/- 0.62 vs. 3.95 +/- 0.77, P <0.01) compared to healthy controls. At rest, mean rBV was lower in HCM patients (0.094 +/- 0.016 vs. 0.138 +/- 0.014 ml.ml-1), and during hyperaemia (0.104 +/- 0.018 ml.ml-1 vs. 0.185 +/- 0.024 ml.ml-1) (all P <0.001) as compared to healthy controls. beta tended to be higher in HCM at rest (9.4 +/- 4.6 vs. 7.7 +/- 4.2 ml.min-1) and hyperaemia (25.8 +/- 6.4 vs. 23.1 +/- 6.2 ml.min-1) as compared to healthy controls. Endo-to-epi MBF decreased during hyperaemia (0.86 +/- 0.15 to 0.64 +/- 0.18, P <0.01) in the septum and (0.92 +/- 0.19 to 0.77 +/- 0.23, P <0.01) in the lateral wall. The rBV was inversely correlated to left ventricular (LV) mass index (P <0.05). Hyperaemic and endo-to-epi MBF were inversely correlated with LV end-diastolic pressure, LV mass index, and LV outflow tract pressure gradient (all P <0.05). MCE-derived MBF correlated well with PET at rest (r =0.84) and hyperaemia (r =0.87), (all P <0.001). CONCLUSIONS: In HCM patients, the blunted hyperaemic perfusion and MFR are due to exhausted autoregulation of myocardial microcirculation as well extravascular compression forces. LV end-diastolic pressure, LV outflow tract pressure gradient, and LV mass index are independent predictors of rBV and hyperaemic MBF. These findings may have important practical implications for the understanding and follow-up of patients with HCM and can be measured accurately with MCE.

Abstract: Background The Frank-Starling law describes the relation between left ventricular volume and function. However, little studies described the relation between left atrial volume (LAV) and function. Aim To describe a LA Frank-Starling law by studying changes in LAV measured by real-time three-dimensional echocardiography (RT3DE). Methods In seventy patients LAV was calculated by RT3DE at end-systole (Vmax), end-diastole (Vmin) and pre-atrial contraction (Vpre-A). According to Vmax, patients were classified into three groups: Vmax <50 ml (Group I), Vmax 50 to 70 ml (Group II), and Vmax >70 ml (Group III). Calculated indices of LA pump function were active atrial stroke volume (AA-SV) defined as Vpre-A inverted exclamation markV Vmin and active atrial emptying fraction (AA-EF), defined as AA-SV / Vpre-A x 100% Results AA-SV increased from Group I to Group II (8.2,,4.9 vs. 19.0,,b 9.2 ml, P <0.0001), but (non-significantly) decreased from Group II to Group III (19.0,,b 9.2 vs. 16.7,,b 12.5 ml). AA-SV was well correlated with Vpre-A (r = 0.56, P <0.0001), but decreased with larger Vpre-A. AA-EF tended to increase from Group I to Group II (33.2,,b 17.5 vs. 43.1,,b 18.2, P < 0.10), but decreased from Group II to Group III (43.1,,b 18.2 vs. 26.2,,b 18.5, P <0.01). Conclusion A Frank-Starling mechanism could be described by RT3DE and evidenced by an increase in LA contractility in response to an increase in LA preload up to a point, beyond which LA contractility decreased.

Abstract: AIMS: The aim of the present study was to assess the relative prognostic value of coronary flow reserve (CFR) and diabetes mellitus (DM) in patients with suspected coronary artery disease (CAD). METHODS: We prospectively studied 347 inhospital patients with chest pain. Coronary angiography was performed in 281 patients (81%). All patients underwent a transthoracic echocardiographic study to evaluate left ventricular function and a stress vasodilator transoesophageal echocardiographic study to evaluate simultaneously CFR and the degree of aortic atherosclerosis (AA). The primary outcome of the study was cardiovascular mortality. RESULTS: During a mean follow-up of 41+/-12 months, 22 patients suffered cardiovascular death. Diabetic patients had a significantly higher AA grade and tended to have a lower CFR and more often significant CAD. Patients with normal CFR had less often significant CAD and tended to have less often DM. Significant univariable predictors of cardiovascular survival were DM, LV end-diastolic diameter, CFR and AA grade. Multivariable regression analysis showed that only CFR (hazard ratio (HR) 2.9, P=0.01) and diabetes (HR 3.1, P=0.01) were independent predictors of cardiovascular survival. CONCLUSIONS: CFR and DM evaluations offer complementary information during vasodilator stress TEE testing. Patients with reduced CFR (impaired microcirculatory function) and DM have the worst prognosis.

Abstract: BACKGROUND: Noncompaction cardiomyopathy (NCCM) is a recently recognized disorder frequently associated with systolic and diastolic heart failures. This study was designed to examine aortic stiffness in NCCM patients and to compare these results to age- and gender-matched controls. METHODS: A total of 20 patients with typical echocardiographic features of NCCM (age 38 +/- 16 years, eight males) were investigated. Their results were compared to 20 age- and gender-matched controls. All subjects underwent a complete two-dimensional transthoracic echocardiographic examination. Systolic (SD) and diastolic (DD) ascending aortic diameters were recorded in M-mode at a level of 3 cm above the aortic valve from a parasternal long-axis view. Aortic stiffness index (beta) was calculated as a characteristic of aortic elasticity, as ln(SBP/DBP)/[(SD - DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, respectively, and ln is the natural logarithm. RESULTS: The number of noncompacted segments in the NCCM patients was 4.6 +/- 2.0. NCCM patients had significantly increased left ventricular dimensions and reduced left ventricular ejection fraction. Compared to controls, aortic stiffness index (beta) was significantly increased in NCCM patients (8.3 +/- 5.2 vs. 3.5 +/- 1.1, p < 0.001). CONCLUSION: Increased aortic stiffness can be observed in patients with NCCM with moderate to severe heart failure. These alterations may be due to neurohormonal changes in heart failure.

Abstract: Cardiac data in adults with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS syndrome) or asymptomatic gene carriers with the mitochondrial deoxyribonucleic acid adenine-to-guanine point mutation at nucleotide pair 3243 are scarce. Twelve subjects (mean-age 35 +/- 13 years), 8 with MELAS syndrome (patients) and 4 asymptomatic gene carriers (carriers), were enrolled in the study. Each subject underwent electrocardiography, exercise testing, Holter monitoring, echocardiography, and genetic and biochemical analysis for respiratory chain enzyme activity (complex I rest activity) in skeletal muscle. On electrocardiography and Hotter monitoring, none of the subjects had evidence of preexcitation, cardiac arrhythmias, or conduction abnormalities. Patients had significantly lower (42 +/- 17% from normal vs 103 +/- 14%, p < 0.02) exercise tolerance. All but 1 of the patients and none of the gene carriers had ragged red fibers on muscle biopsy. The mean percentage of gene mutation in skeletal muscle tended to be higher in patients (53 +/- 19%, range 19% to 73%) compared with carriers (33 +/- 20%, range 15% to 62%). Mean complex I rest activity in patients (36 +/- 18%, range 10% to 58%) was significantly (p < 0.01) lower compared with carriers (120 +/- 60%, range 72% to 205%). Left ventricular (LV) abnormalities were confined to patients with MELAS syndrome. Two patients had LV hypertrophy, 5 had LV systolic abnormalities, and 5 had LV diastolic dysfunction. Apart from 1 patient with an isolated LV diastolic abnormality, all patients with LV abnormalities had ragged red fibers. Patients with abnormal systolic LV function had a trend toward a higher percentage of mutated skeletal muscle (59.7 +/- 10.7% vs 35.8 +/- 21.3%, p < 0.10) and significantly lower complex I rest activity (26.7 +/- 14.0% vs 97.8% +/- 57.9, p < 0.01). In conclusion, none of the MELAS gene carriers had cardiac abnormalities, whereas most patients with the MELAS phenotype, particularly those with ragged red fibers, had LV involvement. (c) 2007 Elsevier Inc. All rights reserved.

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Abstract: Dobutamine stress echocardiography (DSE) has good diagnostic accuracy for the diagnosis of coronary artery disease (CAD). However, in most published diagnostic studies, patients are predominantly men. In women, diagnostic accuracy may be lower because of a lower prevalence and extent of CAD, a higher incidence of dobutamine stress-induced hypotension (resulting in less stress or even nondiagnostic test results), smaller left ventricular chamber size, and the beneficial effects of estrogens on the induction of myocardial ischemia. To determine the diagnostic accuracy of DSE in women, 14 diagnostic studies published through 2006 were identified through a Medline search. For a total of 901 patients, the weighted mean sensitivity and specificity were 72% and 88%, respectively. In 7 studies directly comparing results in women and men, conflicting results were reported. However, pooled data showed nearly identical values for sensitivity and specificity in women and men. Additionally, in 6 studies directly comparing DSE results in women with those of stress nuclear scintigraphy, DSE was as sensitive and more specific to detect CAD (90% vs 70%, p <0.0001). The excellent specificity of DSE in women was also confirmed by excellent normalcy rates, ranging from 92% to 100% in women, with a <5% pretest probability of CAD. In conclusion, despite some theoretical limitations, DSE has reasonable sensitivity and excellent specificity for the detection of CAD in women. Considering the diagnostic problems of exercise electrocardiography and nuclear scintigraphy in women, stress echocardiography may be the stress modality of choice in women because of its superior diagnostic specificity.

Abstract: BACKGROUND: The tricuspid valve (TV) is a complex structure. Unlike the aortic and mitral valve it is not possible to visualize all TV leaflets simultaneously in one cross-sectional view by standard two-dimensional echocardiography (2DE) either transthoracic or transesophageal due to the position of TV in the far field. AIM: Quantitative and qualitative assessment of the normal TV using real-time 3-dimensional echocardiography (RT3DE). METHODS: RT3DE was performed for 100 normal adults (mean age 30 +/- 9 years, 65% males). RT3DE visualization was evaluated by 4-point score (1: not visualized, 2: inadequate, 3: sufficient, and 4: excellent). Measurements included TV annulus diameters (TAD), TV area (TVA), and commissural width. RESULTS: In 90% of patients with good 2DE image quality, it was possible to analyse TV anatomy by RT3DE. A detailed anatomical structure including unique description and measurement of tricuspid annulus shape and size, TV leaflets shape, and mobility, and TV commissural width were obtained in majority of patients. Identification of each TV leaflet as seen in the routine 2DE views was obtained. CONCLUSION: RT3DE of the TV is feasible in a large number of patients. RT3DE may add to functional 2DE data in description of TV anatomy and providing highly reproducible and actual reality (anatomical and functional) measurements.

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Abstract: Aortic stiffness is an important predictor of cardiovascular morbidity and mortality. Non-invasive measurement of aortic stiffness is a promising challenge for echocardiography. The most important [imitation of previous studies was that regional differences for aortic stiffness were not taken into consideration. In our patient, we demonstrated the usefulness of real-time three-dimensional echocardiography in assessment of regional aortic stiffness. (C) 2006 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.

Abstract: Background: Recent technical developments have resulted in high-resolution real time three-dimensional echocardiography (RT3DE). The purpose of this study was to investigate the beneficial role of parasternal-acquired images in addition to apical-acquired images during contrast stress RT3DE. Methods: The study comprised 30 consecutive patients (52 +/- 11 years, 18 males) with chest pain referred for routine stress testing. The contrast RT3DE images were acquired from the apical and parasternal window with a Sonos 7500 echo system attached to a X4 matrix array transducer. Results: From the apical and parasternal acquisition, 464 segments (91%)and 267 segments (52%) could be analyzed, respectively (P < 0.001). From the apical window, more basal segments were not analyzable (22 of 180, 12% vs. 24 of 330, 7%; P = 0.06). From the parasternal window, more apical segments were not analyzable (117 of 150, 78% vs. 126 of 360, 35%; P < 0.01). The mean image quality index of the 464 analyzable segments from the apical-acquired images was 2.43. Fourteen of 180 basal segments (8%), 12 of 180 midventricular segments (7%) and 2 of 150 apical segment (1%) were only available with parasternal data acquisition. In addition to these 28 segments, 79 segments (15%) already visualized from the apical window improved in quality. The overall mean image quality index, now assessed from 492 (96%) of all segments, using both the apical and parasternal acquired data, improved to 2.74 (P < 0.05). Conclusions: Addition of parasternal to apical acquisition of contrast RT3DE data can decrease the number of nonvisualized segments and improve mean image quality.

Abstract: Background: The Frank -Starling law describes the relation between left ventricular volume and function. However, only a few studies have described the relation between left atrial volume (LAV) and function. Objective: To describe an LA Frank -Starling law by studying changes in LAV measured by real-time, threedimensional echocardiography (RT3DE). Methods: LAV was calculated by RT3DE in 70 patients at end-systole (LAV(max)), end-diastole (LAV(min)) and pre-atrial contraction (LAV(pre)-A). According to LAV(max), patients were classified into three groups: LAV(max),50 ml (group I), LAV(max) 50 -70 ml (group II) and LAV(max)>70 ml (group III). Calculated indices of LA pump function were active atrial stroke volume (SV), defined as LAV(pre)-(A) -LAV(min), and active atrial emptying fraction (EF), defined as active atrial SV/ LAV(pre)-(A) 6100% Results: Active atrial SV was significantly higher in group II than in group I (mean (SD) 19.0 (9.2) vs 8.2 (4.9) ml, p, 0.0001), in group III it was non-significantly lower than in group II (16.7 (12.5) vs 19.0 (9.2) ml). Active atrial SV correlated well with LAVpre-A (r = 0.56, p, 0.001), but decreased with larger LAV(pre)-(A). Active atrial EF tended to be higher in group II than in group I (43.1 (18.2) vs 33.2 (17.5), p, 0.10), in group III it was significantly lower than in group II (26.2 (18.5) vs 43.1 (18.2), p, 0.01). Conclusion: A Frank -Starling mechanism in the left atrium could be described by RT3DE, shown by an increase in LA contractility in response to an increase in LA preload up to a point, beyond which LA contractility decreased.

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Abstract: Aim Assessment of pulmonary valve (PV) and right ventricular outflow tract (RVOT) using real-time 3-dimensional echocardiography (RT3DE). Methods Two-dimensional echocardiography (2DE) and RT3DE were performed in 50 patients with congenital heart disease (mean age 32 +/- 9.5 years, 60% female). Measurements were obtained at parasternal views: short axis (PSAX) at aortic valve level and long axis (PLAX) with superior tilting. RT3DE visualization was evaluated by 4-point score (1: not visualized, 2: inadequate, 3: sufficient, and 4: excellent). Diameters of PV annulus (PVAD), and RVOT (RVOTD) were measured by both 2DE and RT3DE, while areas (PVAA) and (RVOTA) by RT3DE only. Results By RUDE, PV was visualized sufficiently in 68% and RVOT excellently in 40%. PVAD and PVAA were measured in 88%. RVOTD and PVAD by 2DE at PLAX were significantly higher than PSAX (P < 0.0001) and lower than that by RUDE (P < 0.001). Conclusion RT3DE helps in RVOT and PV assessment adding more details supplemental to 2DE.

Abstract: The study included 30 patients with hypertrophic cardiomyopathy (HCM) (obstructive and nonobstructive) and 15 control subjects. End-diastolic mitral annulus area (MAA(3D)) and mitral valve area (MVA3D) were measured by real-time 3-dimensional (3D) echocardiography. MVA,2D and peak mitral inflow A wave velocity (V) were measured by 2-dimensional (2D) echocardiography. Left atrial ejection force (LA-EF) was calculated by 2D echocardiography and real-time 3D echocardiography using the formula: 0.5 X 1.06 X (MAA or MVA) X V-2, where (1.06) is blood viscosity. LA-EF2D-MVA, LA-EF3D-MVA, LA-EF3D-MAA, and V were significantly higher in patients with HCM than control subjects (P < .001). LA-FF2D-MVA and LA-EF3D-MVA were lower than LA-EF3D-MAA in HCM only (P < .001). In obstructive HCM, LA-EF2D-MVA, LA-EF2D-MVA, LA-EF3D-MAA, and V were significantly higher than in nonobstructive HCM (P < .05). Left ventricular outflow tract gradient contributed independently to high LA-EF in obstructive HCM. We concluded that HCM is associated with higher LA, EF than normal, and higher in obstructive HCM than nonobstructive indicating a higher atrial workload that is reflected by LA-EF3D-MAA.

Abstract: Dobutamine stress echocardiography is an accepted tool for the diagnosis of coronary artery disease. Some investigators have claimed that 3-dimensional imaging improves the diagnostic accuracy of dobutamine stress echocardiography. The purpose of the present investigation was to examine the role of contrast echocardiography in the improvement of segmental quality and interobserver agreement during stress real-time 3-dimensional echocardiography (RUDE). The study comprised 36 consecutive patients with stable chest pain referred for routine stress testing. Three-dimensional images were acquired with an RUDE system with an X4 matrix-array transducer. All available reconstructed 2-dimensional segments were graded as optimal, good, moderate, or poor. Wall motion was scored as normal, mild hypokinesia, severe hypokinesia, akinesia, or dyskinesia. At peak stress, 466 of the 612 segments (76%) could be analyzed during conventional RUDE. With contrast-enhanced RUDE, the number of available segments increased to 553 (90%). The image quality index during conventional RUDE was 2.2, whereas with contrast-enhanced RUDE, it was 3.1. With conventional RUDE, 2 independent observers agreed on the diagnosis of myocardial ischemia in 85 of 108 coronary territories (79%, kappa = 0.26). With contrast-enhanced RUDE, agreement increased to 95 of 108 coronary territories (88%, kappa = 0.59). Study agreement on myocardial ischemia was present in 26 of 36 studies (72%, kappa = 0.43) with conventional RUDE and in 32 of 36 studies (89%, kappa = 0.77) with contrast-enhanced RUDE. In conclusion, during stress RUDE, contrast-enhanced imaging significantly decreases the number of poorly visualized myocardial segments and improves interobserver agreement for the diagnosis of myocardial ischemia. (c) 2007 Elsevier Inc. All rights reserved.

Abstract: Aim To detect the accuracy of real-time three-dimensional echocardiography (RT3DE) and two-dimensional echocardiography (2DE) for tricuspid annulus (TA) assessment compared with magnetic resonance imaging (MRI). Methods Thirty patients (mean age 34 +/- 13 years, 60% males) in sinus rhythm were examined by MRI, RT3DE, and 2DE for TA assessment. End-diastolic and end-systolic TA diameter (TAD) and TA fractional shortening (TAFS) were measured by RT3DE, 2DE, and MRI. End-diastolic and end-systolic TA area (TAA) and TA fractional area changes (TAFAC) were measured by RT3DE and MRI. End-diastolic and end-systolic right ventricular (RV) volumes and ejection fraction (RV-EF) were measured by MRI. Results The TA was clearly delineated in all patients and visualized as an oval-shaped by RT3DE and MRI. There was a good correlation between TAD(MRI) and TAD(3D) (r = 0.75, P = 0.001), while TAD(2D) was fairly correlated with TAD(3D) and TAD(MRI) (r = 0.5, P = 0.01 for both). There were no significant differences between RT3DE and MRI in TAD, TAA, TAFS, and TAFAC measurements, while TAD(2D) and TAFS(2D) were significantly underestimated (P < 0.001). TAFS(2D) was not correlated with RV-EF, while TAFS(3D) and TAFAC(3D) were fairly correlated with RV-EF (r = 0.49, P = 0.01, and r = 0.47, P = 0.02 respectively). Conclusion RT3DE helps in accurate assessment of TA comparable to MRI and may have an important implication in the TV surgical decision-making processes. RT3DE analysis of TA function could be used as a marker of RV function.

Abstract: Background The tricuspid valve (TV) is a complex structure. Unlike the aortic and mitral valve it is not possible to visualize all TV leaflets simultaneously in one cross-sectional view by standard two-dimensional echocardiography (2DE) either transthoracic or transesophageal due to the position of TV in the far field. Aim Quantitative and qualitative assessment of the normal TV using real-time 3-dimensional echocardiography (RT3DE). Methods RT3DE was performed for 100 normal adults (mean age 30 +/- 9 years, 65% males). RT3DE visualization was evaluated by 4-point score (1: not visualized, 2: inadequate, 3: sufficient, and 4: excellent). Measurements included TV annulus diameters (TAD), TV area (TVA), and commissural width. Results In 90% of patients with good 2DE image quality, it was possible to analyse TV anatomy by RT3DE. A detailed anatomical structure including unique description and measurement of tricuspid annulus shape and size, TV leaflets shape, and mobility, and TV commissural width were obtained in majority of patients. Identification of each TV leaflet as seen in the routine 2DE views was obtained. Conclusion RT3DE of the TV is feasible in a large number of patients. RT3DE may add to functional 2DE data in description of TV anatomy and providing highly reproducible and actual reality (anatomical and functional) measurements.

Abstract: We evaluated the value of baseline parameters derived from tissue Doppler imaging (TDI) for event prediction in patients with heart failure (HF) secondary to ischemic and nonischemic cause who underwent cardiac resynchronization therapy (CRT). Seventy-four consecutive patients with HF (mean age 59 +/- 11 years) underwent CRT. Baseline clinical parameters included New York Heart Association class, 6-minute walking distance, HF cause, and diabetes. TDI-derived parameters included lateral and septal E/E' ratios defined as peak early left ventricular (LV) filling velocity (E wave) to TDI-derived peak early diastolic velocity of the mitral annulus (E' wave). During a median follow-up of 720 days, 21 patients (28%) had cardiac death or hospitalization for HF. These patients more often had an ischemic cause (p <0.05), diabetes (p <0.05), and restrictive filling (p <0.001), less often had LV dyssynchrony (p <0.05), and had higher septal and lateral E/E' ratios (p <0.001 for the 2 comparisons). In a multivariable model using a forward selection algorithm, only the lateral E/E' ratio remained an independent predictor of cardiac outcome. After 3 months of CRT, TDI-derived systolic mitral annular systolic and diastolic velocities improved significantly in nonischemic patients for the septal and lateral sides. In contrast, in ischemic patients no significant improvements were seen. Significant improvements were seen in septal and lateral E/E' ratios in ischemic and nonischemic patients. However, the improvement in lateral E/E' ratio was significantly less and absolute 3-months E/E' ratios were worse in ischemic patients. In conclusion, baseline lateral E/E' ratio is an independent predictor for cardiac events in patients with HF treated with CRT. The worse clinical outcome in ischemic patients may be due to failure of improvement in systolic and diastolic mitral annular velocities after CRT, resulting in a less pronounced improvement in LV filling pressures as demonstrated by this E/E' ratio.

Abstract: Aortic stiffness is an important predictor of cardiovascular morbidity and mortality. Non-invasive measurement of aortic stiffness is a promising challenge for echocardiography. The most important limitation of previous studies was that regional differences for aortic stiffness were not taken into consideration. In our patient, we demonstrated the usefulness of real-time three-dimensional echocardiography in assessment of regional aortic stiffness.

Abstract: AIM: Assessment of pulmonary valve (PV) and right ventricular outflow tract (RVOT) using real-time 3-dimensional echocardiography (RT3DE). METHODS: Two-dimensional echocardiography (2DE) and RT3DE were performed in 50 patients with congenital heart disease (mean age 32 +/- 9.5 years, 60% female). Measurements were obtained at parasternal views: short axis (PSAX) at aortic valve level and long axis (PLAX) with superior tilting. RT3DE visualization was evaluated by 4-point score (1: not visualized, 2: inadequate, 3: sufficient, and 4: excellent). Diameters of PV annulus (PVAD), and RVOT (RVOTD) were measured by both 2DE and RT3DE, while areas (PVAA) and (RVOTA) by RT3DE only. RESULTS: By RT3DE, PV was visualized sufficiently in 68% and RVOT excellently in 40%. PVAD and PVAA were measured in 88%. RVOTD and PVAD by 2DE at PLAX were significantly higher than PSAX (P < 0.0001) and lower than that by RT3DE (P < 0.001). CONCLUSION: RT3DE helps in RVOT and PV assessment adding more details supplemental to 2DE.

Abstract: The study included 30 patients with hypertrophic cardiomyopathy (HCM) (obstructive and nonobstructive) and 15 control subjects. End-diastolic mitral annulus area (MAA(3D)) and mitral valve area (MVA(3D)) were measured by real-time 3-dimensional (3D) echocardiography. MVA(2D) and peak mitral inflow A wave velocity (V) were measured by 2-dimensional (2D) echocardiography. Left atrial ejection force (LA-EF) was calculated by 2D echocardiography and real-time 3D echocardiography using the formula: 0.5 x 1.06 x (MAA or MVA) x V2, where (1.06) is blood viscosity. LA-EF(2D-MVA), LA-EF(3D-MVA), LA-EF(3D-MAA), and V were significantly higher in patients with HCM than control subjects (P < .001). LA-EF(2D-MVA) and LA-EF(3D-MVA) were lower than LA-EF(3D-MAA) in HCM only (P < .001). In obstructive HCM, LA-EF(2D-MVA), LA-EF(3D-MVA), LA-EF(3D-MAA), and V were significantly higher than in nonobstructive HCM (P < .05). Left ventricular outflow tract gradient contributed independently to high LA-EF in obstructive HCM. We concluded that HCM is associated with higher LA-EF than normal, and higher in obstructive HCM than nonobstructive indicating a higher atrial workload that is reflected by LA-EF(3D-MAA).

Abstract: BACKGROUND: Cardiac involvement in mucopolysaccharidosis type I (MPS I) has been studied primarily in its most severe forms. Cardiac involvement, particularly left ventricular (LV) systolic and diastolic function, in the attenuated form of MPS I is less well known. METHODS: Cardiac function was prospectively investigated in 9 adult patients with the attenuated form of MPS I. All patients underwent 12-lead electrocardiography, 24 h Holter monitoring and two-dimensional echocardiography including tissue Doppler imaging (TDI). Eighteen age- and sex-matched healthy volunteers served as a control group. RESULTS: Aortic, mitral and tricuspid valve thickening was seen in, respectively, 5 (56%), 4 (44%) and 2 (22%) patients. Moderate mitral valve stenosis was seen in 1 patient and moderate aortic stenosis in 2 patients. All patients had mild-to-moderate aortic and mitral valve regurgitation and 6 patients (67%) had mild-to-moderate tricuspid valve regurgitation. Despite normal LV dimensions, ejection fraction and mass index, MPS patients had lower mean systolic mitral annular velocities (6.1 +/- 0.6 vs 9.1 +/- 1.4 cm/s, p < 0.01) compared to normal control subjects. Similarly, mean early diastolic mitral annular velocities were lower in MPS patients (7.8 +/- 0.9 vs 13.3 +/- 3.3 cm/s, p < 0.01). CONCLUSION: MPS I patients with the attenuated phenotype have not only valvular abnormalities but also LV diastolic and systolic abnormalities.

Abstract: OBJECTIVE: We sought to assess mitral annular (MA) size and function in hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) using real-time 3-dimensional (3D) echocardiography (RT3DE). METHODS: The study included 30 patients with HCM, 20 patients with DCM, and 30 control subjects. RT3DE measurements included end-systolic and end-diastolic MA area (MAA) (MAA(3D)), MA diameter(3D), MA fractional area change (MAFAC), and MA fractional shortening. In subgroup of 50 patients, magnetic resonance imaging (MRI) was used for MAA(MRI) and MA diameter(MRI) measurement. RESULTS: End-diastolic MAA(3D) was larger in HCM than in control group (P < .0001). Higher MAFAC and MA fractional shortening were present in HCM than in control group (P = .001 and P = .006, respectively). End-systolic and end-diastolic MAA(3D) in DCM were higher than in HCM and control groups (P < .0001). Lower MAFAC and MA fractional shortening were present in DCM than in HCM and control groups (P < .0001). MAFAC correlated well with left ventricular function in control subjects (r = 0.94, P < .0001), whereas correlation was less in DCM (r = 0.53, P = .02) and HCM (r = 0.42, P < .01). RT3DE and MRI measurements were comparable. CONCLUSION: RT3DE assessment of MA size and function in control subjects and patients with cardiomyopathy is accurate and well correlated with MRI.

Abstract: OBJECTIVE: We evaluated two different commercially available real-time 3-dimensional echocardiographic semiautomated border detection algorithms for left ventricular (LV) volume analysis in patients with cardiomyopathy and distorted LV geometry. METHODS: A total of 53 patients in sinus rhythm with various types of cardiomyopathy (mean age 56 +/- 11 years, 28 men) and adequate 2-dimensional image quality were included. The real-time 3-dimensional echocardiographic multiplane interpolation (MI) and full volume reconstruction (FVR) methods were used for LV volume analysis. Magnetic resonance imaging was used as the reference method. RESULTS: A strong correlation (R(2) > 0.95) was found for all LV volume and ejection fraction measurements by either real-time 3-dimensional echocardiographic method. Analysis time was shorter with the FVR method (6 +/- 2 vs 15 +/- 4 minutes, P < .01) as compared with the MI method. Bland-Altman analysis showed greater underestimation of end-diastolic and end-systolic volumes by MI compared with FVR. For the MI method a bias of -24.0 mL (-15.0% of the mean) for end-diastolic volume and -11.3 mL (-18.0% of the mean) for end-systolic volume was found. For FVR analysis these values were -9.9 mL (-6.0% of the mean) and -5.0 mL (-9.0% of the mean), respectively. Ejection fraction was similar for the MI and FVR method with a mean difference compared with magnetic resonance imaging of 0.6 (1.0%) and 0.8 (1.3%), respectively. CONCLUSION: In patients with cardiomyopathy, distorted LV geometry, and good 2-dimensional image quality, the FVR method is faster and more accurate than the MI method in assessment of LV volumes.

Abstract: Objective. We sought to assess mitral annular (MA) size and function in hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (HCM) using real-time 3-dimensional (3D) echocardiography (RT3DE). Methods: The study included 30 patients with HCM, 20 patients with DCM, and 30 control subjects. RT3DE measurements included end-systolic and end-diastolic MA area (MAA) (MAA(3D)), MA diameter(3D), MA fractional area change (MAFAC), and MA fractional shortening. In subgroup of 50 patients, magnetic resonance imaging (MRI) was used for MAA(MRI). and MA diameter(MRI) measurement. Results. End-diastolic MAA(3D) was larger in HCM than in control group (P < .0001). Higher MAFAC and MA fractional shortening were present in HCM than in control group (P =.001 and P =.006, respectively). End-systolic and end-diastolic MAA(3D),, in DCM were higher than in HCM and control groups (P < .0001). Lower MAFAC and MA fractional shortening were present in DCM than in HCM and control groups (P < .0001). MAFAC correlated well with left ventricular function in control subjects (r = 0.94, P < .0001), whereas correlation was less in DCM (r = 0.53, P =.02) and HCM (r = 0.42, P < .01). RT3DE and MRI measurements were comparable. Conclusion: RT3DE assessment of MA size and function in control subjects and patients with cardiomyopathy is accurate and well correlated with MRI.

Abstract: Background Mitral annulus assessment is of great importance for the diagnosis and treatment of mitral valve disease. The present study sought to assess the value of real-time three-dimensional echocardiography for the assessment of true mitral annulus diameter (MAD). Methods One hundred and fifty patients (mean age 38 +/- 18 years) with adequate two-dimensional (2D) echocardiographic image quality underwent assessment of MAD(2D) and MAD(3D) (with real-time three-dimensional echocardiography). In a subgroup of 30 patients true MAD was validated with magnetic resonance imaging (MRI). Results There was a good interobserver agreement for MAD(2D) (mean difference = -0.25 +/- 2.90 mm, agreement: -3.16, 2.66) and MAD(3D) (mean difference = 0.29 +/- 2.03, agreement = -1.74, 2.32). Measurements of MAD(2D) and MAD(3D) were well correlated (R = 0.81, P < 0.0001). However, MAD(3D) was significantly larger than MAD(2D) (3.7 +/- 0.9 vs. 3.3 +/- 0.8 cm, P < 0.0001). In the subgroup of 30 patients with MRI validation, MAD(3D) and MAD(MRI) were significantly larger than MAD(2D) (3.3 +/- 0.5 and 3.4 +/- 0.5 cm vs. 2.9 +/- 0.4 cm, both P < 0.001). There was no significant difference between MAD(MRI) and MAD(3D). Conclusion MAD(3D) can be reliably measured and is superior to MAD(2D) in the assessment of true mitral annular size.

Abstract: METHODS: The study included 25 hypertrophic cardiomyopathy (HCM) patients (15 non-obstructive and 10 obstructive) and 25 controls for assessment of left atrial (LA) volume, mass and function by two-dimensional echocardiography. Measurement included mean LA diameter (LAD), LA mass = {(mean LAD + anterior LA wall + posterior LA wall)(3) - mean LAD(3)} x 0.8 + 0.6, LA volume = [(8/3 pi L . A1 . A2), where L is LA length, A1 and A2 are LA area in 4-chambers and 2-chambers, respectively] including maximum (V (max)), minimum (V (min)), and pre-atrial contraction (V (pre-A)), total atrial stroke volume (TA-SV), TA emptying fraction (TA-EF), active atrial SV (AA-SV), AA-EF, passive atrial SV (PA-SV), PA-EF, atrial expansion index (AEI), and LA kinetic energy (LA-KE) = (1/2) x AA-SV x P x V(2). RESULTS: LAD, LA mass, V (max), V (min), and V (pre-A) were significantly higher in HCM than controls. TA-SV and TA-EF were comparable in both HCM subgroups and controls. AA-SV and LA-KE were significantly higher in both HCM subgroups than controls. LA-KE was significantly higher in obstructive HCM than non-obstructive (P < 0.001). PA-EF and AEI were significantly lower in obstructive HCM than controls (P < 0.05). CONCLUSION: HCM is associated with increased LA size and augmented LA pump function especially obstructive type. LA conduit and reservoir functions are impaired in obstructive HCM.

Abstract: Cardiac data in adults with mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS syndrome) or asymptomatic gene carriers with the mitochondrial deoxyribonucleic acid adenine-to-guanine point mutation at nucleotide pair 3243 are scarce. Twelve subjects (mean age 35 +/- 13 years), 8 with MELAS syndrome (patients) and 4 asymptomatic gene carriers (carriers), were enrolled in the study. Each subject underwent electrocardiography, exercise testing, Holter monitoring, echocardiography, and genetic and biochemical analysis for respiratory chain enzyme activity (complex I rest activity) in skeletal muscle. On electrocardiography and Holter monitoring, none of the subjects had evidence of preexcitation, cardiac arrhythmias, or conduction abnormalities. Patients had significantly lower (42 +/- 17% from normal vs 103 +/- 14%, p <0.02) exercise tolerance. All but 1 of the patients and none of the gene carriers had ragged red fibers on muscle biopsy. The mean percentage of gene mutation in skeletal muscle tended to be higher in patients (53 +/- 19%, range 19% to 73%) compared with carriers (33 +/- 20%, range 15% to 62%). Mean complex I rest activity in patients (36 +/- 18%, range 10% to 58%) was significantly (p <0.01) lower compared with carriers (120 +/- 60%, range 72% to 205%). Left ventricular (LV) abnormalities were confined to patients with MELAS syndrome. Two patients had LV hypertrophy, 5 had LV systolic abnormalities, and 5 had LV diastolic dysfunction. Apart from 1 patient with an isolated LV diastolic abnormality, all patients with LV abnormalities had ragged red fibers. Patients with abnormal systolic LV function had a trend toward a higher percentage of mutated skeletal muscle (59.7 +/- 10.7% vs 35.8 +/- 21.3%, p <0.10) and significantly lower complex I rest activity (26.7 +/- 14.0% vs 97.8% +/- 57.9, p <0.01). In conclusion, none of the MELAS gene carriers had cardiac abnormalities, whereas most patients with the MELAS phenotype, particularly those with ragged red fibers, had LV involvement.

Abstract: BACKGROUND: Aortic valve stenosis (AS) is the most frequent form of valvular heart disease. The number of studies evaluating the effect of aortic valve replacement (AVR) for AS on aortic vascular function is limited. The aim of the present study was to examine alterations in aortic distensibility in patients with AS during a 1-year follow-up after AVR. METHODS: Twelve patients with severe AS who underwent AVR were prospectively examined (mean age 65 +/- 11 years, 7 men). Systolic and diastolic ascending aortic diameters (SD and DD, respectively) were recorded in M mode 3 cm above the aortic valve from a parasternal long-axis view. The SD and DD were measured at the time of maximum anterior motion of the aorta and at the start of the QRS complex, respectively. Aortic stiffness index (beta) was defined as [ln(SBP/DBP)] x DD/deltaD, where ln is the natural logarithm, SBP and DBP are the systolic and diastolic blood pressure values, respectively, and deltaD = SD - DD. RESULTS: As expected, aortic stenosis severity and left ventricular mass decreased significantly after AVR. Aortic diameter changes (systolic minus diastolic dimensions) progressively increased and the aortic stiffness index progressively improved to levels comparable with those of age-, sex-, and risk factor-matched controls at the 1-year assessment. CONCLUSIONS: Aortic valve replacement in patients with AS is associated with a progressive improvement in aortic distensibility to 1-year values similar to those of controls.

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Abstract: Background Aortic valve stenosis (AS) is the most frequent form of valvular heart disease. The number of studies evaluating the effect of aortic valve replacement (AVR) for AS on aortic vascular function is limited. The aim of the present study was to examine alterations in aortic distensibility in patients with AS during a 1-year follow-up after AVR. Methods Twelve patients with severe AS who underwent AVR were prospectively examined (mean age 65 I I years, 7 men). Systolic and diastolic ascending aortic diameters (SD and DD, respectively) were recorded in M mode 3 cm,above the aortic valve from a parasternal long-axis view. The SD and DD were measured at the time of maximum anterior motion of the aorta and at the start of the QRS complex, respectively. Aortic stiffness index () was defined as [ln(SBP/DBP)] x DD/AD, where In is the natural logarithm, SBP and DBP ore the systolic and diastolic blood pressure values, respectively, and Delta D = SD - DD. Results As expected, aortic stenosis severity and left ventricular mass decreased significantly after AVR. Aortic diameter changes (systolic minus diastolic dimensions) progressively increased and the aortic stiffness index progressively improved to levels comparable with those of age-, sex-, and risk factor-matched controls at the 1-year assessment. Conclusions Aortic valve replacement in patients with AS is associated with a progressive improvement in aortic distensibility to 1-year values similar to those of controls.

Abstract: Dobutamine stress echocardiography (DSE) has good diagnostic accuracy for the diagnosis of coronary artery disease (CAD). However, in most published diagnostic studies, patients are predominantly men. In women, diagnostic accuracy may be lower because of a lower prevalence and extent of CAD, a higher incidence of dobutamine stress-induced hypotension (resulting in less stress or even nondiagnostic test results), smaller left ventricular chamber size, and the beneficial effects of estrogens on the induction of myocardial ischemia. To determine the diagnostic accuracy of DSE in women, 14 diagnostic studies published through 2006 were identified through a Medline search. For a total of 901 patients, the weighted mean sensitivity and specificity were 72% and 88%, respectively. In 7 studies directly comparing results in women and men, conflicting results were reported. However, pooled data showed nearly identical values for sensitivity and specificity in women and men. Additionally, in 6 studies directly comparing DSE results in women with those of stress nuclear scintigraphy, DSE was as sensitive and more specific to detect CAD (90% vs 70%, p < 0.0001). The excellent specificity of DSE in women was also confirmed by excellent normalcy rates, ranging from 92% to 100% in women, with a < 5% pretest probability of CAD. In conclusion, despite some theoretical limitations, DSE has reasonable sensitivity and excellent specificity for the detection of CAD in women. Considering the diagnostic problems of exercise electrocardiography and nuclear scintigraphy in women, stress echocardibgraphy may be the stress modality, of choice in women because of its superior diagnostic specificity. (c) 2007 Elsevier Inc. All rights reserved.

Abstract: Ultrasound contrast has shown to improve endocardial border definition. The purpose of this study was to evaluate the value of contrast agent-enhanced versus non-contrast agent-enhanced real-time 3-dimensional echocardiography (RT3DE) for the assessment of left ventricular (LV) volumes and ejection fraction. Thirty-nine unselected patients underwent RT3DE with and without SonoVue contrast agent enhancement and magnetic resonance imaging (MRI) on the same day. An image quality index was calculated by grading all 16 individual LV segments on a scale of 0 to 4: 0, not visible; 1, poor; 2, moderate; 3, good; and 4, excellent. The 3-dimensional data sets were analyzed offline using dedicated TomTec analysis software. By manual tracing, LV end-systolic volume, LV end-diastolic volume, and LV ejection fraction were calculated. After contrast agent enhancement, mean image quality index improved from 2.4 +/- 1.0 to 3.0 +/- 0.9 (p <0.001). Contrast agent-enhanced RT3DE measurements showed better correlation with MRI (LV end-diastolic volume, r = 0.97 vs 0.86; LV end-systolic volume, r = 0.96 vs 0.94; LV ejection fraction, r = 0.94 vs 0.81). The limits of agreement (Bland-Altman analysis) showed a similar bias for RT3DE images with and without contrast agent but with smaller limits of agreement for contrast agent-enhanced RT3DE. Also, inter- and intraobserver variabilities decreased. In a subgroup, patients with poor to moderate image quality showed an improvement in agreement after administration of contrast agent (+/- 24.4% to +/- 12.7%) to the same level as patients with moderate to good image quality without contrast agent (+/- 10.4%). In conclusion, contrast agent- enhanced RT3DE is more accurate in assessment of LV function as evidenced by better correlation and narrower limits of agreement compared with MRI, as well as lower intra- and interobserver variabilities. (C) 2007 Elsevier Inc. All rights reserved.

Abstract: AIMS: The current study sought to assess if pre-implantation lateral-to-septal delay (LSD) > or =60 ms assessed by spectral pulsed-wave myocardial tissue Doppler imaging (PW-TDI) could predict successful long-term outcome after cardiac resynchronization therapy (CRT). METHODS AND RESULTS: Sixty patients (72% males, mean age 59 +/- 10 years) who were referred for CRT according to the ACC/ESC guidelines were enrolled in the study. All patients underwent spectral PW-TDI before and 1 year after CRT. Two left ventricular (LV) dyssynchrony time intervals, T(O) and T(P) (time to onset and peak of LV myocardial velocity, respectively), LSD were recorded. Left ventricular dyssynchrony was defined as LSD > or =60 ms. Clinical response was defined as an improvement in >1 NYHA class plus improvement in 6-min walk distance (6MWD) > or =25%, echocardiographic response was defined as a > or =15% reduction in LV end-systolic volume (LV-ESV). One year after CRT, 50 patients (83%) were clinical responders and 47 patients (78%) were echocardiographic responders. Both T(O) and T(P) LV dyssynchrony indices failed to predict echocardiographic CRT outcome. In addition, there were no significant differences between 'synchronous' and 'dyssynchronous' patient populations at baseline or follow-up in either clinical (NYHA class and 6MWD) or echocardiographic (LV ejection fraction, LV end-diastolic, and end-systolic) variables. CONCLUSION: The great majority of patients referred for CRT benefit clinically from it. However, spectral PW-TDI failed to predict CRT outcome. When PW-TDI dyssynchrony was applied for selection of proper CRT patients, up to 80-86% of the patients with synchronous LSD that had proven clinical and echocardiographic benefit from CRT would have been denied CRT.

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Abstract: Cardiac hydatid disease or echinococcosis is a rare complication of Echinococcus granulosus infection. Traditionally, the diagnosis is made with two-dimensional transthoracic echocardiography. This case report shows the complementary function of the different echo modalities (two-dimensional, three-dimensional, and contrast echocardiography) and MRI in the evaluation of pericardial hydatid cyst.

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Abstract: Cardiac hydatid disease or echinococcosis is a rare complication of Echinococcus granulosus infection. Traditionally, the diagnosis is made with two-dimensional transthoracic echocardiography. This case report shows the complementary function of the different echo modalities (two-dimensional, three-dimensional, and contrast echocardiography) and MRI in the evaluation of pericardial hydatid cyst.

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Abstract: BACKGROUND: Pompe's disease, also known as acid maltase deficiency or glycogen storage disease type II, is an autosomal recessive disorder in which deficient activity of the enzyme acid alpha-glucosidase causes intra-lysosomal accumulation of glycogen in muscle and other tissues. The current study was designed to assess aortic stiffness index (beta), as a characteristic of aortic elasticity during transthoracic echocardiography in patients with Pompe's disease. METHODS: A total of 17 patients (age 44+/-8 years, 5 males) with Pompe's disease were studied. Their results were compared to 17 age- and gender-matched controls. In all patients, the ascending aorta was recorded with M-mode echocardiography. beta was calculated as ln(SBP/DBP)/[(SD-DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, SD and DD are the systolic and diastolic aortic diameters, and 'ln' is the natural logarithm. RESULTS: Diastolic aortic diameter was 27.4+/-2.4 mm in Pompe patients and 25.6+/-2.7 mm in controls (P<0.05). Systolic aortic diameters did not differ between the groups (29.4+/-2.5 mm vs 28.3+/-2.4 mm, P=ns). Aortic stiffness index (beta) was increased in Pompe patients compared to controls (14.6+/-10.1 vs 5.1+/-2.6, P<0.001). CONCLUSIONS: The results of this study indicate that aortic stiffness is increased in patients with Pompe's disease. This may be due to glycogen storage in the vessel wall causing reduced vascular elasticity.

Abstract: BACKGROUND: Mitral annulus assessment is of great importance for the diagnosis and treatment of mitral valve disease. The present study sought to assess the value of real-time three-dimensional echocardiography for the assessment of true mitral annulus diameter (MAD). METHODS: One hundred and fifty patients (mean age 38 +/- 18 years) with adequate two-dimensional (2D) echocardiographic image quality underwent assessment of MAD(2D) and MAD(3D) (with real-time three-dimensional echocardiography). In a subgroup of 30 patients true MAD was validated with magnetic resonance imaging (MRI). RESULTS: There was a good interobserver agreement for MAD(2D) (mean difference = -0.25 +/- 2.90 mm, agreement: -3.16, 2.66) and MAD(3D) (mean difference = 0.29 +/- 2.03, agreement = -1.74, 2.32). Measurements of MAD(2D) and MAD(3D) were well correlated (R = 0.81, P < 0.0001). However, MAD(3D) was significantly larger than MAD(2D) (3.7 +/- 0.9 vs. 3.3 +/- 0.8 cm, P < 0.0001). In the subgroup of 30 patients with MRI validation, MAD(3D) and MAD(MRI) were significantly larger than MAD(2D) (3.3 +/- 0.5 and 3.4 +/- 0.5 cm vs. 2.9 +/- 0.4 cm, both P < 0.001). There was no significant difference between MAD(MRI) and MAD(3D). CONCLUSION: MAD(3D) can be reliably measured and is superior to MAD(2D) in the assessment of true mitral annular size.

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