What's New in Arrhythmogenic Cardiomyopathies.

Arrhythmogenic Cardiomyopathy (ACM) is a hereditary cardiomyopathy often presenting with sudden cardiac death (SCD) in young athletic individuals [...]

The relationship between functional capacity and ultrasonic tissue characterization in patients with idiopathic dilated cardiomyopathy

Background: Ultrasonic tissue characterization (UTC) has been widely used to investigate left ventricular (LV) dysfunction in various cardiac disorders. The aim of this study was to investigate the correlation between functional capacity and UTC in patients with idiopathic dilated cardiomyopathy (IDCM).Methods and Results: Treadmill test according to modified-Bruce protocol was performed in 48 patients with IDCM to assess their functional capacity. Baseline clinical and echocardiographic variables were obtained and UTC was performed on images obtained from septum and posterior wall (PW). Cyclic variation (CV) index of mean gray level (MGL) was calculated according to the formula: [(MGLdiastole − MGLsystole) ÷ MGLdiastole] × 100.PW and septum CV indices were correlated with exercise duration (r = 0.63, p = 0.001 and r = 0.67, p = 0.0001, respectively) and “MET” level (r = 0.80, p = 0.0001 and r = 0.83,p = 0.0001, respectively). The ROC curve analysis revealed that the PW CV index was a strong indicator of good exercise capacity (> 8 METs) with an AUC of 0.97 (95% CI0.90–1.0), as the interventricular septum (IVS) CV index (AUC = 0.97, 95% CI 0.89–1.0). Sensitivity, specificity, positive predictive value, and negative predictive value to identify good exercise capacity for IVS CV index were 90%, 88%, 82%, and 94%, respectively and for the PW CV index, 90%, 88%, 82%, and 94%, respectively.Conclusions: In this particular study, we found out that in patients with severe LV dysfunction good exercise capacity was related to septum and PW CV indices measured by UTC, and these indices may be used as an indirect prognostic marker in heart failure

Cardiomyopathy related desmocollin-2 prodomain variants affect the intracellular cadherin transport and processing

BACKGROUND Arrhythmogenic cardiomyopathy can be caused by genetic variants in desmosomal cadherins. Since cardiac desmosomal cadherins are crucial for cell-cell-adhesion, their correct localization at the plasma membrane is essential. METHODS Nine desmocollin-2 variants at five positions from various public genetic databases (p.D30N, p.V52A/I, p.G77V/D/S, p.V79G, p.I96V/T) and three additional conserved positions (p.C32, p.C57, p.F71) within the prodomain were investigated in vitro using confocal microscopy. Model variants (p.C32A/S, p.V52G/L, p.C57A/S, p.F71Y/A/S, p.V79A/I/L, p.I96l/A) were generated to investigate the impact of specific amino acids. RESULTS We revealed that all analyzed positions in the prodomain are critical for the intracellular transport. However, the variants p.D30N, p.V52A/I and p.I96V listed in genetic databases do not disturb the intracellular transport revealing that the loss of these canonical sequences may be compensated. CONCLUSION As disease-related homozygous truncating desmocollin-2 variants lacking the transmembrane domain are not localized at the plasma membrane, we predict that some of the investigated prodomain variants may be relevant in the context of arrhythmogenic cardiomyopathy due to disturbed intracellular transport

<em>Two-dimensional thermal analysis of liquid hydrogen tank insulation</em>

Liquid hydrogen (LH2) storage has the advantage of high volumetric energy density, whileboil-off losses constitute a major disadvantage. To minimize the losses, complicatedinsulation techniques are necessary. In general, Multi Layer Insulation (MLI) and a Vapor-Cooled Shield (VCS) are used together in LH2 tanks. In the design of an LH2 tank with VCS,the main goal is to find the optimum location for the VCS in order to minimize heatleakage. In this study, a 2D thermal model is developed by considering the temperaturedependencies of the thermal conductivity and heat capacity of hydrogen gas. The developedmodel is used to analyze the effects of model considerations on heat leakagepredictions. Furthermore, heat leakage in insulation of LH2 tanks with single and doubleVCS is analyzed for an automobile application, and the optimum locations of the VCS forminimization of heat leakage are determined for both cases.</p

Assembly line rebalancing and worker assignment considering ergonomic risks in an automotive parts manufacturing plant

This paper recommends a new kind of assembly line rebalancing and worker assignment problem, taking ergonomic risks into account. Assembly line rebalancing problem (ALRBP) occurs when a current line must be rebalanced due to conditions such as changes in demand, production processes, product design, or quality issues. Although there are several research attempts on ALRBP in the relevant literature, only a few studies consider workers as unique individuals. This paper aims to solve the double reassignment problem: tasks to workers and workers to stations, considering ergonomic risk factors. This paper is the first study that comprises worker assignment and ergonomic constraints in ALRBP literature to the best of our knowledge. Objectives of our novel problem are to minimize rebalancing cost, which includes transportation of tasks and workers and minimize stations' ergonomic risk factors. A randomized constructive rule-based heuristic approach is developed to cope with the problem. The proposed solution approach is applied to benchmark data, and obtained results are promising. Moreover, the proposed solution approach is implemented in an automotive parts manufacturing plant. (c) 2022 by the authors; licensee Growing Science, Canad

Flash pulmonary edema: A rare cause and possible mechanisms

AbstractFlash pulmonary edema frequently develop in case of bilateral renal artery stenosis and unilateral renal artery stenosis with functional solitary kidney. In some rare cases, unilateral renal artery stenosis with bilaterally functional kidneys may also lead to flash pulmonary edema. Here, we present a case of flash pulmonary edema caused by accessory renal artery stenosis. To our knowledge, it is the first case reported in the literature

A detailed review and analysis of assembly line rebalancing problems

Purpose - Various approaches and algorithms have been proposed since the 1950s to solve the assembly line (AL) balancing problem. These methods have established an AL configuration from the beginning. However, a prebalanced AL may have to be rebalanced in real life for many reasons, such as changes in the cycle time, production demand, product features or task operation times. This problem has increasingly attracted the interest of scientists in recent years. This study aims to offer a detailed review of the assembly line rebalancing problems (ALRBPs) to provide a better insight into the theoretical and practical applications of ALRBPs