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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. [ABSTRACT FROM AUTHOR]

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. [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]

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]

Fabry disease (α-galactosidase A deficiency) is an X-linked recessive lysosomal storage disease in which left ventricular hypertrophy (LVH) is common, and if severe, may mimic hypertrophic obstructive cardiomyopathy. Alcohol-induced percutaneous transluminal septal myocardial ablation (PTSMA) has been used as a safe and effective method to alleviate LVH obstruction in patients with hypertrophic obstructive cardiomyopathy (HCM). We describe a case of a classically affected Fabry 53-year-old male with symptomatic HCM (NYHA class III with exertional angina) who was treated with PTSMA. The procedure safely and effectively alleviated symptomatic left ventricular outflow tract obstruction at long-term follow-up, and the patient's NYHA classification was reduced to NYHA class I to II. (ECHOCARDIOGRAPHY, Volume 22, April 2005) [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]