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Aim of the Study: To assess the diagnostic utility of a simplified approach to relative apical sparing of longitudinal strain (RAS LS) using only an apical four-chamber view (A4C) in patients with AL amyloid cardiomyopathy (ALAC)., Methods: We retrospectively evaluated echocardiographic recordings of 20 patients with ALAC, 20 patients with Fabry disease-related cardiomyopathy (FD), and 20 patients with concentric hypertensive left ventricular hypertrophy (HLVH) matched for mean LV mean thickness. Peak segmental LS values of the interventricular septum and lateral LV wall were measured in the A4C using two-dimensional speckle-tracking echocardiography. RAS LS was calculated as average apical LS/(average basal LS + average midventricular LS)., Results: Relative apical sparing of longitudinal strain values in patients with ALAC (1.23 ± 0.64) were significantly higher than those in FD patients (0.75 ± 0.19, P < 0.05) as well as in individuals with HLVH (0.75 ± 0.23, P < 0.05), but with a significant overlap. The optimal RAS LS value differentiating ALAC from FD and HLVH with 70% sensitivity and 75% specificity was 0.88 (AUC 0.79). In multivariate modeling, RAS LS was significantly additive to traditional predictors of ALAC (low QRS voltage and pseudoinfarct ECG patterns, pericardial effusion, E/e' ratio, E-wave deceleration time; P < 0.05 for all models)., Conclusions: Simplified RAS LS evaluation represents an attractive approach for diagnostics of ALAC. However, because of considerable overlap with other disorders with hypertrophic phenotype, the analysis of RAS LS in the A4C should be combined with other traditional echocardiographic and ECG predictors in differentiating ALAC from other forms of concentric LV wall thickening., (© 2018 Wiley Periodicals, Inc.)

Background: Fabry cardiomyopathy (FC) and light-chain amyloid cardiomyopathy (AL) present with concentric left ventricular (LV) hypertrophy/remodeling and diastolic rather than systolic dysfunction. Direct comparisons are difficult due to rarity and confounded by variability of LV thickness., Aims: To compare LV diastolic and systolic properties between patients with FC and AL in a cohort matched for interventricular septal thickness (IVS)., Methods: A two-center echocardiographic analysis was performed, comprising 118 patients with IVS ≥12 mm (FC and AL 59 patients each) matched by IVS., Results: Fabry cardiomyopathy patients had larger LV end-diastolic diameter (47.7 [44.0-50.9] vs 45.0 [41.5-49.0] mm, P = 0.002), better LV ejection fraction (EF 68.7 [63.4-74.0] vs 63.0 [54.0-70.0]%, P = 0.001) and midwall fractional shortening (midFS 14.8 [13.0-16.1] vs 12.1 [8.9-15.0]%, P = 0.006). LV EF <40% was rare in both (2% vs 7%, P = 0.17). AL patients expressed higher LV diastolic dysfunction grade (III in 26% vs 4%, II in 21% vs 12% and I in 54% vs 84%, P = 0.004), with higher E/e' ratio (13.6 [10.2-18.8] vs 9.8 [7.5-12.3], P < 0.0001). Average E/e' ratio and midFS were significantly associated with NYHA severity in both groups (P < 0.05 for all)., Conclusions: Matched AL patients had worse LV diastolic function than FC, driven by E/e'. Significant LV systolic dysfunction was rare overall. MidFS and E/e' were associated with heart failure severity in both groups., (© 2018 Wiley Periodicals, Inc.)

Background: The non-invasive assessment of amyloid heart disease may be challenging. Cardiac magnetic resonance (CMR) represents a method of choice for assessment of left ventricular (LV) morphology and function, and it also provides a unique possibility to evaluate the presence of amyloid deposition by the late gadolinium enhancement (LGE) technique. However, so far, published studies have not been consistent in terms of described LGE patterns associated with amyloid cardiomyopathy., Aims: To compare echocardiographic and CMR assessment of LV morphology and function and to evaluate the presence and pattern of LGE in a population of patients with AL amyloid cardiomyopathy., Methods: Twenty-two consecutive patients with newly diagnosed AL amyloid cardiomyopathy and without contraindications to CMR were comprehensively examined by echocardiography and CMR., Results: Echocardiography and CMR did not differ in the evaluation of interventricular septal thickness, LV end-diastolic diameter and ejection fraction. Significant differences were found between echocardiographic and CMR estimates of LV end-diastolic volume (P<0·01) and LV mass (P<0·001). Various global LGE patterns (transmural homogenous or heterogeneous, subendocardial) were present in 17 patients (77%), patchy LGE was observed in one case (4·5%) and suboptimal nulling of the myocardium was reported in two subjects (9%)., Conclusions: Echocardiography significantly overestimates LV mass and underestimates LV volumes in patients with AL amyloid cardiomyopathy as compared to CMR. As it is present in more than three quarters of individuals with AL amyloid cardiomyopathy, any type of global LGE pattern may be considered as pathogenomic for amyloid heart disease., (© 2014 Scandinavian Society of Clinical Physiology and Nuclear Medicine. Published by John Wiley & Sons Ltd.)

Background: Left atrial (LA) enlargement is a predictor of worse outcome after catheter ablation for atrial fibrillation (AF). Widely used two-dimensional (2D)-echocardiography is inaccurate and underestimates real LA volume (LAV). We hypothesized that baseline clinical characteristics of patients can be used to adjust 2D-ECHO indices of LAV in order to minimize this disagreement., Methods: The study enrolled 535 patients (59 ± 9 years; 67% males; 43% paroxysmal AF) who underwent catheter ablation for AF in three specialized centers. We investigated multivariately the relationship between 2D-echocardiographic indices of LA size, specifically LA diameter in M-mode in the parasternal long-axis view (LAD), LAV assessed by the prolate-ellipsoid method (LAVEllipsoid), LAV by the planimetric method (LAVPlanimetry), and LAV derived from 3D-electroanatomic mapping (LAVCARTO)., Results: Cubed LAD of 106 ± 45 ml, LAVEllipsoid of 72 ± 24 ml and LAVPlanimetry of 88 ± 30 ml correlated only modestly (r = 0.60, 0.69, and 0.53, respectively) with LAVCARTO of 137 ± 46 ml, which was significantly underestimated with a bias (±1.96 standard deviation) of -31 (-111; +49) ml, -64 (-132; +2) ml, and -49 (-125; +27) ml, respectively; p < 0.0001 for their mutual difference. LA enlargement itself, age, gender, type of AF, and the presence of structural heart disease were independent confounders of measurement error of 2D-echocardiographic LAV., Conclusion: Accuracy and precision of all 2D-echocardiographic LAV indices are poor. Their agreement with true LAV can be significantly improved by multivariate adjustment to clinical characteristics of patients.

The aim of the study was to describe right ventricular (RV) structural and functional changes in Fabry disease (FD). A detailed echocardiographic examination was performed in 58 patients with proven FD (mean age 40 +/- 16 years, 24 men). RV hypertrophy (RVH) was present in 40% of affected subjects with similar prevalence in both genders. Approximately two thirds of patients with left ventricular hypertrophy (LVH) also exhibited RVH. RV dilatation was not present in any subject. RV systolic dysfunction was noted in only 1 female subject. RV diastolic dysfunction was present in 47% of 45 subjects in whom RV filling was assessed. RV diastolic dysfunction was associated with the presence of RVH. A significant correlation between RV wall thickness and age (r = 0.52, P < .001) and left ventricular mass index (r = 0.70, P < .001) was noted. RVH with normal chamber size and preserved systolic but impaired diastolic function represents a typical RV structural change in FD. Its prevalence and degree are related to the prevalence and degree of LVH and the age of the patient.

Background: Takotsubo cardiomyopathy consists of reversible systolic left ventricular apical ballooning associated with chest pain. Electrocardiographic abnormalities and the minimal rise of serum cardiac markers are similar to those in acute myocardial infarction, but without evidence of myocardial ischemia or injury. To date, many reports concerning this kind of acute reversible heart failure have been published, but the information available about the management of affected patients is scarce and the clinical data are incomplete., Aims: In the present study, we report a collection of 40 patients who were affected by Takotsubo cardiomyopathy obtained in a multicentric international study, aiming to investigate the origins, and the clinical and instrumental patterns, and to establish the best diagnostic criteria for this syndrome., Methods and Results: In the analysed group, the mean age was 68 years, of whom 85% were women. On admission to hospital, 68% of patients reported chest pain. An electrocardiogram (ECG) showed anterolateral (34%) or anterior (36%) ST segment elevation. The ECG demonstrated hyperkinesis of the basal segments with a severe hypokinesis of the other segments. Mean ejection fraction was 42.53%. Three patients died within the first 24 h from acute heart failure. The remaining 37 patients showed a complete resolution of symptoms and a complete normalization of the kinesis deficiency. Sixteen patients underwent myocardial scintigraphy, nine cases underwent myocardial biopsy and two patients received an ergonovine test., Conclusion: Our results demonstrate a good course of Takotsubo cardiomioathy, after the initial phase. An echocardiogram is an important tool for improving the diagnosis.

Background: Systemic sclerosis (SSc) is an important cause of pulmonary arterial hypertension (PAH), with an estimated prevalence of 7.85-26.7%. Objective: Our aim was to estimate the prevalence of PAH among patients with SSc in the Czech Republic and to compare haemodynamics in SSc patients diagnosed with PAH through screening with those diagnosed previously, based on symptoms. Methods: During 2007, SSc patients in the Czech Republic, without significant pulmonary function impairment or cardiac disease, underwent screening for PAH with transthoracic echocardiography. Those with a tricuspid regurgitant (TR) jet gradient suggestive of PAH (>30 mmHg) underwent subsequent right heart catheterisation (RHC) to confirm the diagnosis (mean pulmonary arterial pressure, mPAP, ≥25 mmHg; pulmonary capillary wedge pressure, ≤15 mmHg). Haemodynamics in patients diagnosed with PAH in this way were compared with those in patients diagnosed previously, based on symptoms. Results: Two hundred and three SSc patients (mean age, 53.8 ± 13 years; 82.3% women) from 26 rheumatology practices were screened. Among these, 17 had a TR jet gradient >30 mmHg and underwent RHC; PAH was confirmed in six patients. These six patients were found to have significantly lower mPAP than nine patients diagnosed previously with PAH, based on symptoms (31.17 ± 5.56 vs. 46.89 ± 9.48 mmHg, p = 0.0014). Conclusion: Prevalence of PAH in our SSc cohort was 7.08%. SSc patients diagnosed with PAH through screening have less advanced disease in terms of haemodynamics than those with PAH diagnosed previously based on symptoms; their prognosis is therefore likely to be more favourable. [ABSTRACT FROM AUTHOR]

Purpose: To assess cardiac structural and functional changes induced by competitive amateur cycling. Methods: Fifty-one young competitive amateur male cyclists and 47 age- and gender-matched control subjects underwent complex transthoracic echocardiographic examination focused on the detailed assessment of cardiac size and function, especially of the left ventricle (LV). Subsequently, spiroergometry was performed in all study participants and its results were compared to echocardiographic data. Results: Higher left ventricular mass indexes due to the greater LV wall thickness and LV end-diastolic diameter were found in amateur cyclists as compared to control subjects. There were no differences with regard to the LV systolic function parameters. However, significantly better indices of LV diastolic function were present in cyclists. A significant correlation between maximal oxygen pulse and LV mass index/height2.7, LV diastolic parameters and right ventricular size was noted. Conclusions: Amateur competitive cycling leads to considerable LV structural and functional changes. The increases in LV wall thickness and cavity size together with supranormal diastolic properties are key characteristics of this LV remodeling. As well, LV mass, diastolic function, and RV size are predictors of exercise capacity, indicating an important involvement of these elements in improving cardiac function with endurance training. (Echocardiography 2010;27:11-16) [ABSTRACT FROM AUTHOR]

The time interval (TEa–E) between onsets of early diastolic mitral annular velocity (Ea) and early mitral inflow velocity (E) has been proposed as a new index of left ventricular (LV) relaxation. We aimed to assess the influence of preload changes on this parameter by examining 25 healthy volunteers at rest, after passive leg lifting, and after administration of nitroglycerin combined with Valsalva maneuver. To obtain septal and lateral TEa–E, the time intervals between peak R-wave on electrocardiogram and the onsets of Ea and E were measured in different cardiac cycles. E and Ea were significantly changed by preload manipulations. We did not observe significant changes of TEa–E intervals after leg lifting or after nitroglycerin administration combined with Valsalva maneuver (P = NS). TEa–E was < 0 ms in several subjects using either septal or lateral Ea recordings. We conclude that TEa–E may be load independent in subjects with normal LV systolic and diastolic function as compared to other Doppler parameters. Therefore, this index could be potentially used as a reliable noninvasive parameter of LV relaxation. Nevertheless, even slight differences in cardiac cycle lengths might influence the measurements of TEa–E interval. For that reason, the clinical use of TEa–E seems to be significantly limited because of a wide margin of error in its measurement due to the inability to obtain Doppler recordings during the same cardiac cycle. [ABSTRACT FROM AUTHOR]

Background: Available studies of the effect of hemodialysis (HD) on left ventricular (LV) performance brought ambiguous results. Therefore we aimed to investigate the effect of acute preload reduction induced by HD on conventional and novel parameters of LV structure and function. Methods: Thirty-six patients underwent echocardiography 1 hour prior to and 1 hour following regular HD. M-mode, two-dimensional, and Doppler echocardiography were used to analyze conventional LV structural and functional parameters. Systolic and diastolic mitral annular velocities assessed by pulsed-wave tissue Doppler echocardiography (PW-TDE) and flow propagation velocity (Vp) of early LV inflow were measured as novel indices of LV systolic and diastolic function. Results: After HD, all heart chambers including LV significantly reduced in their size. The reduction in LV mass was also observed. Parameters of LV systolic function—ejection fraction and systolic mitral annular velocity, significantly improved, whereas fractional shortening did not. As for LV diastolic function, conventional Doppler parameters and Vp were substantially changed after HD. Conversely, PW-TDE diastolic velocities were not significantly affected. Conclusions: The fluid removal induced by HD leads to a substantial decrease in LV size and mass. The improvement of LV longitudinal contraction documented by PW-TDE seems to be responsible for the increase in global LV systolic function after HD. While standard Doppler parameters of LV diastolic function and Vp are significantly affected by preload reduction, PW-TDE diastolic indices appears to be less load dependent. Therefore, PW-TDE represents a promising method for the LV diastolic function assessment in patients on HD. [ABSTRACT FROM AUTHOR]

Background: Pulsed wave tissue Doppler echocardiography (PW-TDE) and color M-mode are new Doppler methods for left ventricular (LV) diastolic function assessment. To date, few studies have compared the data obtained by these methods in the same series of patients and compared them to the current clinical reference method of detecting LV diastolic function.Aims: To determine the utility of PW-TDE and color M-mode parameters in the assessment of LV diastolic function in the typical patient population encountered in daily clinical practice and to compare their discriminating power.Methods: Early diastolic septal mitral annular velocity (Em) determined by PW-TDE and color M-mode flow propagation velocity (Vp) were measured in 86 male patients and compared to LV filling patterns obtained using standard Doppler indices. Values of Em<0.08 m s−1 and Vp<0.5 m s−1 were considered as markers of abnormal LV diastolic function.Results: A value of Em<0.08 m s−1 distinguished mild to moderate LV diastolic dysfunction with higher sensitivity and specificity than Vp<0.5 m s−1 (96% and 87% vs. 73% and 84%, respectively). A comparison of receiver operating characteristic curves showed a significant difference for areas under the curve in favor of Em (p<0.01). In a stepwise multiple logistic regression analysis, a pseudonormal filling pattern and an EF > 60% were identified as significant predictors of Vp false negative results (p<0.05).Conclusions: Em appears to be superior to Vp in the detection of mild to moderate LV diastolic dysfunction. Vp failed to detect abnormal LV diastolic function in particular in patients with preserved LV systolic function and a pseudonormal filling pattern type. [ABSTRACT FROM AUTHOR]

Abstract: Left ventricular (LV) hypertrophy is the hallmark of cardiac involvement in Fabry disease (FD). However, its pathogenesis is not clearly understood as pathologic substrate accumulation represents only 1–2% of the total cardiac mass. Abnormal myocardial energy metabolism has been previously demonstrated in different forms of cardiomyopathies. We hypothesized that myocardial energy status at the time of diagnosis could have a relationship to gain of LV mass in FD. In the group of 16 affected subjects, the indicators of energetic state of cardiac muscle determined by magnetic resonance spectroscopy showed significant negative correlation with annual increase in LV mass, evaluated during long-term follow-up (8±3 years). Myocardial energy metabolism may therefore represent one of the mechanisms contributing to development of FD-related cardiomyopathy. [ABSTRACT FROM AUTHOR]

Primary cardiac tumors are very rare findings. Papillary fibroelastomas are benign tumors comprising less than 10% of all primary cardiac neoplasms. They are almost always attached to valve surfaces; their location along the ventricular and atrial walls is exceptional. Affected patients are mostly asymptomatic, however peripheral or central embolic events are not rare. We describe an interesting case of papillary fibroelastoma with nonspecific systemic symptoms, arising directly from the left ventricular apex. So far, only four such cases were reported in current literature. [ABSTRACT FROM AUTHOR]

Coronary artery anomalies represent a diverse group of congenital disorders characterized by abnormalities of coronary arteries anatomy. We describe an extremely rare case of giant torturous left circumflex artery draining to the right atrium manifesting by palpitations and atrial fibrillation. [ABSTRACT FROM AUTHOR]

We present images of a papillary fibroelastoma on a pulmonary valve – echocardiography, intraoperative images, macroscopic and microscopic images of the tumor in this uncommon location. Image 1 • Papillary fibroelastomas are rare benign tumors located most commonly on the aortic or mitral valves. • Their surface is furrowed by avascular fronds resembling a sea anemone or a pom-pom. • Avascular fronds are composed predominantly of fibrous tissue and elastic fibers covered by bland endothelium. • Some papillary fibroelastomas can be excised by a valve-sparing approach. [ABSTRACT FROM AUTHOR]

Abstract: Background: Cardiac involvement is responsible for substantial morbidity and mortality in Anderson–Fabry disease (AFD). We sought to document its onset and progression in a population of male and female AFD patients. Methods: We performed a cross sectional echocardiographic study of a cohort of 177 male and female AFD patients with subsequent longitudinal follow-up of 76 patients (38 males and 38 females; mean follow-up 4.5 years) who did not receive enzyme replacement therapy. Results: In this population, aged 3.3 to 70.8 years, a strong correlation between age and left ventricular mass indexed (LVMi, g/m2.7) was found in both males and females (P <0.0001 for both). At the initial examination 48.6% of the male patients and 36.4% of the female patients were classified as having left ventricular hypertrophy (LVH). The cumulative prevalence of LVH peaked at age 40 years in males and 60 years in females. In patients with longitudinal follow-up, LVMi increased by 4.07±1.03 g/m2.7 per year in males and by 2.31±0.81 g/m2.7 in females (P <0.01, Wilcoxon rank sum). In patients with LVH at baseline, the median progression rate was 5.52 g/m2.7 per year in males and by 1.80 g/m2.7 in females (P =0.12). Conclusion: AFD is associated with high prevalence of LVH in both genders. However, the age of onset is delayed in females and progression rate slower. [Copyright &y& Elsevier]