Multi-parametric speckle tracking analyses to characterize cardiac amyloidosis: a comparative study of systolic left ventricular longitudinal myocardial mechanics.

Cardiac amyloidosis (CAM), the most common cardiac storage disease is associated with significant changes in left-ventricular (LV) morphology and function. To gain particular insights into LV systolic longitudinal myocardial mechanics we investigated seven parameters derived by speckle-tracking-echocardiography (STE) in patients with confirmed CAM (n = 59). The results were compared with those of individuals with healthy heart (n = 150) and another primary myocardial disease with also thickened myocardium and severe diastolic and systolic LV-dysfunction (symptomatic LV-non-compaction-cardiomyopathy, LV-NC, n = 30). In addition to standard echocardiographical measures, the STE-derived data were evaluated and documented utilizing polar-diagrams to obtain overviews of longitudinal myocardial mechanics of the entire LV. Compared with healthy individuals, patients with CAM and LV-NC showed significantly reduced LV-ejection-fraction (EF), global longitudinal systolic peak-strain, strain-rate, and displacement. Pre-systolic stretch-index, post-systolic index, and the EF/global peak-longitudinal-strain-ratio (EF/S) were increased. In contrast to healthy-hearts and the LV-NC group only patients with CAM demonstrated significantly reduced time-to-peak systolic longitudinal strain and time-to-peak strain-rate. Although the level of the segmental values in longitudinal mechanics was significantly different between the groups, comparable intraventricular baso-apical parameter-gradients were found for systolic longitudinal peak-strain and strain-rate, pre-systolic-stretch-index, post-systolic-index, and peak systolic displacement. Compared to ATTR-amyloidosis (ATTR-CAM), patients with AL-amyloidosis (AL-CAM) demonstrated significantly lower end-diastolic and end-systolic LV-volumes, LV-mass-indices, relative apical strain, time-to-peak systolic longitudinal strain, and time-to-peak longitudinal strain-rate. CAM and LV-NC demonstrated altered myocardial mechanics with significantly different STE-derived echocardiographical parameters. ATTR-amyloidosis and AL-amyloidosis had at least significantly different time-to-peak strain, time-to-peak strain-rate and relative apical sparing values.

Athletes with valvular heart disease and competitive sports: a position statement of the Sport Cardiology Section of the European Association of Preventive Cardiology.

This article provides an overview of the recommendations from the Sports Cardiology section of the European Association of Preventive Cardiology on sports participation in individuals with valvular heart disease (VHD). The aim of these recommendations is to encourage regular physical activity including sports participation, with reasonable precaution to ensure a high level of safety for all affected individuals. Valvular heart disease is usually an age-related degenerative process, predominantly affecting individuals in their fifth decade and onwards. However, there is an increasing group of younger individuals with valvular defects. The diagnosis of cardiac disorders during routine cardiac examination often raises questions about on-going participation in competitive sport with a high dynamic or static component and the level of permissible physical effort during recreational exercise. Although the natural history of several valvular diseases has been reported in the general population, little is known about the potential influence of chronic intensive physical activity on valve function, left ventricular remodelling pulmonary artery pressure, and risk of arrhythmia. Due to the sparsity of data on the effects of exercise on VHD, the present document is largely based on clinical experience and expert opinion.

Systolic longitudinal global and segmental myocardial mechanics in symptomatic isolated left ventricular non-compaction cardiomyopathy.

BACKGROUND: Left ventricular (LV) non-compaction cardiomyopathy (LV-NC) is rare, and data of segmental myocardial mechanics are largely lacking. We investigated myocardial longitudinal mechanics in adults with symptomatic LV-NC (n = 30) versus individuals with healthy hearts (n = 150). The contribution of compacted and non-compacted myocardial layer to systolic LV function has to be determined. METHODS: Seven parameters derived from speckle tracking echocardiography were evaluated and documented utilizing polar-diagrams to obtain overviews of myocardial mechanics of the entire LV. RESULTS: According to embryonal myocardial development, non-compacted myocardium was mostly located in mid-ventricular and apical segments of the free LV wall. LV ejection fraction was reduced in LV-NC (34 ± 15%, healthy 63 ± 5%, P < .0001). The compact wall layer in LV-NC demonstrated increasing systolic radial thickness (diastolic 5.6 ± 1.4, systolic 6.5 ± 1.4mm, P = .016), whereas the non-compacted layer remained unchanged or tended to decrease in thickness (diastolic 17.6 ± 5.3, systolic 16.0 ± 4.6mm, P = .22). Compared with heart-healthy individuals in LV-NC peak systolic longitudinal strain (healthy -21.1% vs. LV-NC -8.8, P < .0001), peak systolic longitudinal strain-rate (-1.23%/s vs. -0.64, P < .0001), and peak longitudinal displacement (12.1 vs. 5.6 mm, P < .0001) were reduced, while pre-systolic stretch index (1.31% vs. 3.2%, P < .0001) and post-systolic index (2.5% vs. 15.9%, P < .0001) increased. Time-to-peak longitudinal strain (371 vs. 389 ms, P = .065) and time-to-peak longitudinal strain rate (181 vs. 200 ms, P = .0677) did not differ significantly. In LV-NC, there were no significant differences between analyses using an interpolated endocardial border along the edges of the recesses and the endocardial edge of the compact wall layer. Hence, LV function appeared to depend only on the thin compact wall layer. CONCLUSION: In LV-NC, myocardial efficiency is severely diminished compared with healthy controls and LV function seemed to depend mainly on the compact myocardial wall layer.

Unusual Midventricular LV Obstruction With Flow Aimed to the Apex Due to Apical RV Pacing: Elimination by Sequential Septo-Apical RV Pacing.

We report the case of an 88-year-old man who developed a mid-left-ventricular (LV) obstruction caused by apical pacing to manage third-degree atrioventricular block. The flow generated by the obstruction was directed toward the LV apex and appeared from late systole to early diastole. The obstruction appeared to result from earlier contraction at the apex compared with the midventricular portion of the LV, which was followed by an also earlier apical relaxation. The obstruction was eliminated by using a second right-ventricular mid-septally attached electrode combined with a sequential septo-apical pacing (septal stimulation 40 ms before apical pacing).

Physiological left ventricular segmental myocardial mechanics: Multiparametric polar mapping to determine intraventricular gradients of myocardial dynamics.

OBJECTIVE: We investigated physiological systolic left ventricular (LV) myocardial mechanics and gradients to provide a database for later studies of diseased hearts. METHODS: The analyses were performed in 131 heart-healthy individuals and included seven parameters of myocardial mechanics using speckle tracking echocardiography (STE). RESULTS: Basal to apical and circumferentially significant physiological intraventricular parameter gradients of myocardial activity were determined. Global mean values and segmental ranges were peak systolic longitudinal strain -21.2 ± 3.3%, 95% confidence interval [CI] -21.8% to -20.6%), gradient (basal to apical) -16.0% to -26.7%; peak systolic longitudinal strain rate -1.24 ± 0.31%/s, 95% CI -1.29% to -1.19%/s, gradient (basal to apical) -0.91% to -1.61%/s; post-systolic index 2.6 ± 3.2%, 95% CI 3.15%-2.05%, gradient (basal/medial/apical) 7.0/1.2/2.4%; pre-systolic stretch index 1.3 ± 2.7%, 95% CI 1.77%-0.83%, gradient (basal/medial/apical) 6.5/0.2/1.3%; peak longitudinal displacement 12.2 ± 2.6 mm, 95% CI 12.6-11.8 mm, gradient (basal to apical) 21.0-3.4 mm; time-to-peak longitudinal strain 370 ± 43 ms, 95% CI 377-363 ms, gradient (basal to apical) 396-361 ms; and time-to-peak longitudinal strain rate 180 ± 47 ms, 95% CI 188-172 ms, gradient (basal to apical) 150-200 ms. CONCLUSION: This study generated a database of seven STE-derived parameters of physiological segmental and global myocardial LV mechanics. The resulting sets of three-dimensional intraventricular mappings of the entire LV provide physiological parameter gradients in baso-apical and circumferential direction by applying the 17-segment polar model. This will facilitate comparison of systolic myocardial activity of the healthy LV with diseased or otherwise altered (eg, sports) hearts.

Echocardiographically derived effective valve opening area in mitral prostheses: a comparative analysis of various calculations using continuity equation and pressure half time method.

Detection of dysfunctional mitral valve prostheses (MP) remains complex even though being optimized by considering echocardiographically derived prosthetic effective orifice area (VA). The purpose was to compare VA in MP, calculated by the continuity equation (CE) using peak velocities (CEVpeak), mean velocities (CEVmean), velocity-time integrals (CEVTI) and the pressure half time method using 220 ms as constant first (PHT220) as well as optimized constants. In 267 consecutive patients with normally functioning MP, we investigated VA within the first postoperative month. With increasing prosthetic sizes, mean VA values also increase in all calculations. The statistical curves demonstrate no significant difference in graphical steepness but show different levels. Comparison of mean VA showed the known systematic higher values of PHT220 and significantly decreased results when using CEVTI. This systematic difference between mean VA applying PHT220 versus CEVTI is approximately 1.0 cm(2) for all prosthetic sizes. Calculations via CEVpeak were close to the results of CEVTI. CEVmean produced values, which graphically correspond to the PHT220 curve. Only PHT220 detected the constructional equal prosthetic inner ring width between 29 and 31 mm. To compensate the systematic difference between CEVTI and PHT220, an optimized constant of 140 ms was calculated to be applied in PHT (PHT140). VA is a robust and, therefore, preferable parameter for investigating MP. If needed, both CE and PHT are applicable with a systematical difference between CEVTI and PHT220. An optimized constant of 140 ms (PHT140) should be applied when calculating VA of mitral valve prostheses via PHT.

A differentiated morphological parameter-coding system to describe the suitability of mitral valve stenoses intended for percutaneous valvotomy.

Percutaneous balloon-mitral-valvotomy (PBMV) is an alternative to surgery in selected patients with mitral valve (MV) stenosis (MS). Applying echocardiography, suitability for PBMV is assessed by detailed morphological description. Echo-scores alone are suboptimal to describe MV morphology, because single parameters, important for a decision concerning PBMV, are not distinguishable out of a score number. The aim was to design a tool (coding-system), which combines a number for a stenotic MV like scores (for statistical options) and decodable, generally applied parameters describing the MS morphology. The reproducibility of the MS morphology using the coding-system has to be tested in 90 patients. A separate group of 297 patients (pts) with MS, scheduled for PBMV, should be investigated prospectively applying the coding-system and a comparable score. We chose the Wilkins score (WS) as representative of scores. The coding-system is designed as a parameter sequencing set consisting of 6 digits. The first digit indicates a decision code concerning suitability for PBMV. The following 5 digits indicate generally accepted morphological parameters, which are partially also used in the WS. Therefore, the MS morphology can be "read" retrospectively by decoding. 201/297 patients were found suitable for PBMV. Applying the coding-system all 201 suitable patients were correctly distinguished from 96 morphologically unsuitable patients. Astonishingly 48/96 of the rejected patients showed a WS ≤8 whereas 28/201 of the suitable patients demonstrated a WS >8. 25/28 of them showed a successful initial outcome. Applying the generally known threshold of "8" when predicting suitability of a MS, the WS demonstrated an initial success rate of 62 %, sensitivity of 0.87, specificity of 0.45, precision of 0.79, and accuracy of 0.78. Applying the coding-system, the initial success rate was 70.8 %, sensitivity = 0.96, specificity = 1.0, precision = 1.0, and accuracy = 0.97. The coding-system is an advanced diagnostic aid, is statistically applicable, offers a decodable morphological description, includes a decision code regarding suitability for PBMV, and can be used for comparing different groups of patients with MS by calculating "mean morphologies" of groups.

Left ventricular dyssynchrony predicts clinical response to CRT - a long-term follow-up single-center prospective observational cohort study.

BACKGROUND: Until now, there is no consensus regarding the definition of a clinical response to cardiac resynchronization therapy (CRT) in patients with chronic heart failure (CHF) and systolic left ventricular (LV) dysfunction. The aim of this study was to evaluate if echocardiography is predictive for an objective improvement in exercise capacity during long-term follow-up of CRT. METHODS: Each patient underwent echocardiography and spiroergometry both at baseline and at last follow-up. Left ventricular dyssynchrony (LVD) before CRT was defined by tissue Doppler imaging (TDI) as intra-LV delay ≥40 msec (septal-lateral or anterior-posterior). Clinical response to CRT was defined as increase of peakVO2 or as increase of maximal workload >10% as compared to baseline. RESULTS: Mean follow-up was 69 ± 37 months. From the 238 consecutive patients included in the study, 141 (59%) were classified as clinical responders and 97 (41%) as nonresponders. Baseline data of responders and nonresponders were comparable. However, clinical responders showed more often LVD (64%) than nonresponders (42%, P = 0.004). On multivariate regression analysis, nonischemic origin of CHF (ß-coefficient in the final model 0.1, P = 0.04) and LVD at baseline (ß-coefficient in the final model 0.2, P < 0.001) were independently associated with clinical response during long-term follow-up. Patients with LVD at baseline had significant more often an improvement of left ventricular ejection fraction >10% (P = 0.02) and a reduction of left ventricular end-diastolic diameter (LVEDD) >10% (P < 0.01) than patients without LVD at baseline. CONCLUSIONS: LVD at baseline as assessed by a straightforward echocardiographic approach predicts the long-term clinical response to CRT and is associated with a more pronounced reverse LV remodeling.

Myocardial fibrosis severity on cardiac magnetic resonance imaging predicts sustained arrhythmic events in hypertrophic cardiomyopathy.

BACKGROUND: The purpose of our study was to correlate the incidence of adequate implantable cardioverter-defibrillator (ICD) interventions in hypertrophic cardiomyopathy (HCM) patients with risk markers (RMs) for sudden cardiac death (SCD) plus myocardial fibrosis as detected by late gadolinium-enhanced cardiac magnetic resonance (LGE-CMR) imaging. METHODS: In all, 87 patients with HCM underwent LGE-CMR imaging prior to ICD implantation, performed for secondary (n = 2; 2%) or primary SCD prophylaxis (n = 85; 98%). Fibrosis was graded with a 17-segment left ventricular model (0 = absent, 1 = point-shaped, 2 = limited to 1 left ventricular segment, 3 = involving ≥ 2 segments). During follow-up, ICD memories were read out by a physician blinded to the individual patient data. RESULTS: The number of RMs per patient was 1.9 ± 0.8. Myocardial fibrosis was present in 78 patients (90%); 26 (30%) had a fibrosis score of 3. During follow-up (3.5 ± 2.6 [range, 0.2-11.4 years]), 15 patients had 50 appropriate ICD interventions. Episodes of atrial fibrillation were found in 28 patients. Fibrosis severity correlated with occurrence of ventricular tachycardia (Cramér's V, or φc = 0.4, P < 0.001) and atrial fibrillation (φc = 0.6, P < 0.001). On multivariate regression analysis, an independent association between myocardial fibrosis (ß = 0.6, P < 0.01) and sustained ventricular tachycardia was found. CONCLUSIONS: In HCM patients treated with ICD implantation because of a high SCD risk by traditional RM assessment, a high rate of arrhythmic events was observed during long-term follow-up. In a cohort of patients with clinical markers for high risk of SCD, severity of myocardial fibrosis as detected by an easy LGE-CMR scoring system was associated with future arrhythmic events and appropriate ICD therapies.

Marked Regression of Left Ventricular Hypertrophy after Outflow Desobliteration in HOCM.

We present an HOCM patient in whom marked regression of left ventricular hypertrophy occurred within two years following outflow desobliteration by percutaneous septal ablation. Maximum wall thickness (initially documented by both echo and MRI) decreased from 34 mm to 22 mm (followup by echo only due to presence of the ICD), crossing the threshold value of 30 mm which was one of the risk markers that had triggered the primary prophylactic ICD implantation in this case prior to septal ablation.

The importance of training in echocardiography: a validation study using pocket echocardiography.

AIMS: To investigate the training effect in echocardiography by using recent hand-held ultrasound scanners (HANDs). METHODS: In this prospective study, 320 consecutive patients were scanned with HAND by an inexperienced ultrasonographer over a period of 8 weeks. Imaging studies were compared with high-end echocardiography as gold standard. Segmental endocardial border delineation was scored to describe image quality. Assessment of left ventricular dimensions, regional/global left ventricular function and grading of valve disease were compared. RESULTS: A significant reduction in examination duration, improvement in image quality, substantial agreement in functional assessment (κ > 0.61, P < 0.01) and wall motion scoring (κ=0.67, P < 0.01) could be observed over time. The correlation in left ventricular measurements was excellent (r > 0.98, P < 0.01). The detection of valve diseases and pericardial effusions was sufficient, but the grading only moderate (κ < 0.6). CONCLUSION: Well-grounded training in echocardiography leads to a rapid improvement in image acquisition and interpretation over a period of a few weeks. Basic diagnostic findings could be interpreted with high accuracy after short training. Interpretation of complex findings remained difficult. The time needed to carry out an examination using pocket echocardiography could not be reduced to less than 3-4 min per patient. New educational concepts are warranted.

Diagnostic performance of handheld echocardiography for the assessment of basic cardiac morphology and function: a validation study in routine cardiac patients.

AIMS: To investigate the intra- and interrater variability of expert users in the interpretation of handheld echocardiographic studies (HAND). METHODS: We scanned 320 consecutive patients with both HAND and high-end (HIGH) scanners. Images were interpreted independently by two blinded level III echocardiographers. Readings from the HIGH scanner served as the gold standard. Segmental endocardial-border delineation was scored to describe image quality. Assessment of left ventricular (LV) dimensions and regional/global LV function, and grading of valve disease were compared. RESULTS: We obtained correlations of r > 0.8 (P < 0.01) for intrarater variability for both expert readers when they analyzed HAND and HIGH images in relation to image quality, wall-motion abnormalities, and LV measurements. For intrarater variability of LVEF assessment, the correlations were at least moderate (r > 0.6, P < 0.01). Interrater variability for HIGH images was r = 0.9 (P < 0.01) for all parameters. Interrater variability for HAND images was less favorable for all parameters, but was at least moderate (r > 0.6, P < 0.01). All cases of pericardial effusion were detected. The agreement for the detection and grading of mitral and aortic regurgitation was at least moderate (κ > 0.6, P < 0.01). Detection of tricuspid regurgitation was less favorable, but only cases of mild regurgitation were missed. All cases of aortic stenosis were detected by both echocardiographers. CONCLUSIONS: In relation to the basic assessment of cardiac morphology and function, the interpretation by experienced echocardiographers of images obtained using handheld echocardiographic devices showed a moderate to very good correlation with standard echocardiography.

The occurrence of atrial fibrillation in former top-level handball players above the age of 50.

OBJECTIVE: Cardiac adaptation to sports activity in endurance athletes is considerably different from that in power athletes. The effects of a high-level team sport like handball, one of the most popular sports in the world, performed at a younger age, on cardiac rhythm in individuals above the age of 50 have not been investigated to date. METHODS: Thirty-three former top-level handball players from the first German league (6 former world champions and numerous Olympians) (57.5 +/- 5.5 y) joined our screening programme for former athletes and underwent electrocardiography, echocardiography and spiroergometry. Data were compared to 24 sedentary healthy controls. RESULTS: Ten of the 33 athletes suffered from atrial fibrillation (AF). Left ventricular diameter was 53.68 +/- 4.88 mm in the athletes group and 50.58 +/- 4.12 mm in the healthy controls. Analysing the subgroups of handball players ('AF group' and 'non-AF group'), spiroergometry showed oxygen consumption at the anaerobic threshold of 27.54 +/- 6.77 ml/kg/min in the AF group and 31.24 +/- 10.33 ml/kg/min in the non-AF group (P = 0.228). Absolute left atrial diameter was 44.34 +/- 4.41 mm in the AF group (non-AF group 38.94 +/- 3.77 mm, P < 0.001) (healthy controls 37.54 +/- 4.34 mm, compared with all athletes P = 0.015). In all individuals left ventricular wall thickness was within normal limits. However, myocardial walls were thicker in the AF group (11.28 +/- 1.83 mm) than in the non-AF group (9.44 +/- 1.26 mm, P = 0.002). Athletes in the AF group (187.6 +/- 6.42 cm) were significantly taller than in the non-AF group (180.91 +/- 7.31 cm, P = 0.018). CONCLUSION: Not only endurance training, but also sports activity with a relevant static component, like team handball, might predispose for AF above the age of 50. LA size, height and myocardial wall thickness seem to affect the risk of developing AF. More data in non-endurance sports are mandatory to confirm this hypothesis.

Effective valve opening area in the detection of dysfunctional aortic valve prostheses: a differentiated statistical analysis of this parameter including the introduction of minimal expected normal values as borderline to dysfunctional stenotic prostheses.

BACKGROUND: Dysfunction of heart valve prostheses (VP) is a life-threatening complication and the diagnosis remains difficult. The motivation for this study was to improve the detection of dysfunctional VP by optimizing application of the prosthetic effective orifice area (VA). For this reason the minimal expected normal VA (VA(expected)) was introduced. METHODS: We investigated echocardiographically 1,369 normally functioning aortic valve prostheses (AVP). Mean VA, transprosthetic peak (PPG) and mean pressure gradients (MPG) were evaluated to gain reference values depending on prosthetic size and construction principle. Mean VA(expected) was calculated by applying a simple formula that was developed empirically using statistical analyses. The results were compared with those of 65 dysfunctional AVPs. RESULTS: VA(expected) can be applied as a threshold between normal and dysfunctional stenotic AVP and showed a correct estimation in 87% of all normally functioning and 100% of dysfunctional stenotic VPs. The sensitivity for all prosthetic sizes is 1.0, independently of the constructional principle of the VP. Specificity ranged between 0.8 and 1.0, dependent on VP size. The formula representing VA(expected) is simple and can be executed easily. CONCLUSION: As nearly independent of stroke volume and in consideration of VA(expected), VA seems to have become one of the preferable parameters for detecting pathological stenotic AVPs echocardiographically. The additional application of PPG/MPG and other parameters permits prostheses with relevant isolated regurgitation and patient-prosthesis-mismatch to be distinguished.

Myocardial fibrosis is associated with biventricular dysfunction in patients with hypertrophic cardiomyopathy.

AIMS: To assess left (LV) and right ventricular (RV) function by two-dimensional (2D) speckle tracking echocardiography and its relation to myocardial fibrosis in hypertrophic cardiomyopathy (HCM). METHODS: We enrolled 50 HCM patients (30 male; 47.3 ± 9.9 years) in our study. Each patient received echocardiography with modern high-end scanners. For speckle tracking analysis of LV and RV function the dedicated software was used. The presence of myocardial fibrosis was detected by cardiac magnetic resonance imaging (MRI). RESULTS: For intraobserver variability of RV global longitudinal strain, we found a correlation of r = 0.89 (p < 0.001) with a minor bias of 4.9 ± 2.9%. On cardiac MRI 30 patients (60%) demonstrated late gadolinium-enhancement (LGE) of the LV. Of these patients only 7% showed LGE of the RV. HCM patients with myocardial fibrosis had less global longitudinal LV strain in comparison to patients without myocardial fibrosis (-12.8 ± 2.2 vs -21.1 ± 2.6, P < 0.001), thicker interventricular septums (23.7 ± 4.0 vs 19.2 ± 5.1, P < 0.001), larger left atria (34.9 ± 7.1 vs 23.9 ± 5.1, P < 0.001), and impaired diastolic function (E/A-ratio: 1.02 ± 0.22 vs 1.15 ± 0.18, P < 0.01). Comparable results were found for RV function. LV and RV strain correlated with r = 0.85 (p < 0.001). CONCLUSIONS: HCM is not only a disease of the LV. LGE in HCM is associated with both LV and RV dysfunction. Although RV LGE occurs only in a minority of patients with HCM and LV fibrosis, speckle tracking echocardiography is feasible for evaluating LV and RV dysfunction in these patients.