Relevant aspects of golden retriever muscular dystrophy for the study of Duchenne muscular dystrophy in humans

ABSTRACT: Golden Retriever muscular dystrophy (GRMD) is the most representative model for studying Duchenne muscular dystrophy (DMD) in humans, owing its phenotypic expression. DMD is a recessive disorder linked to the X chromosome in which the loss of dystrophin induces progressive weakness and degeneration of the skeletal and cardiac muscles, which lead to replacement by connective and adipose tissues. Onset of clinical signs occurs between 2 and 5 years of age, and many patients die from heart or respiratory failure. The main studies concerning dystrophic Golden Retrievers (DGR) sought to elucidate the pathophysiology of the disease and its clinical implications to develop therapies and alternative treatments to improve the quality of life and increase longevity of DMD patients. This review presents an overview of relevant contributions of the DGR model for elucidating DMD in humans

Influence of peracetic delignification on biomass lignocellulosic complex

The process of delignification of plant biomass with peracetic acid was studied and the effect of the duration of peracetic delignification on the degree of delignification and the degree of removal of the carbohydrate complex in the final cellulose products was established. The treatment of different representatives of plant biomass was performed with peracetic acid at a concentration of 8% and at different time of the process. The study was focused on the process of chemical treatment of six different representatives of non-wood plant raw materials in the form of straw, agricultural waste, as well as stems of fast-growing industrial and fodder crops. Cellulose products were obtained with a yield of about 50% and a whiteness of greater than 60%. It was shown that an increase in the duration of processing naturally leads to a decrease in the yield of the final product, which is associated with the intensive oxidation and removal of lignin, as well as partial destruction of the polysaccharide component of biomass. The investigated method of delignification proved to be effective for the removal of more than 90% of lignin in the entire studied time interval, but the selectivity of the removal of lignin and carbohydrates depended significantly on the type of plant material. By an increase in the efficiency of lignin removal with peracetic acid, the studied plants are located in the following row: sverbiga (bunias orientalis)&gt;shchavnat&gt;lavatera&gt;rapeseed&gt;wheat straw&gt;amaranth. Conducted research work gives grounds to claim that peracetic delignification is an effective method of chemical treatment of plant biomass; it allows obtaining cellulose products that can be used in the production of paper products for various purposes, as well as for the chemical industry.</jats:p

Right ventricular function and systolic pressure effect on survival of patients with tricuspid regurgitation

Abstract   Severe tricuspid regurgitation (TR) is associated with poor outcome. The current analysis investigated the long term outcome of TR patients. Methods Historical retrospective cohort of all cardiovascular patients evaluated at a tertiary heart center between 2007 and 2019. The current analysis included all patients who underwent echocardiographic evaluation. TR severity was extracted for all patients from the echocardiographic reports. Primary outcome was all cause mortality and was available for all patients from the national population register. Results Final cohort included 97,561 subjects, of whom 42,187 (43%) were outpatients. Mean age was 66±17 and 55,976 (57%) were men. Mild, moderate and severe TR was documented in 27,389 (28%), 2,871 (3%) and 1,812 (2%) patients, respectively. During a median follow up of 50 months [IQR 22–83] 18,476 (19%) patients died. Kaplan-Meier survival analysis demonstrated increased risk of death with increasing degree of TR (FIGURE; p Log rank &amp;lt;0.001). Multivariate cox regression with adjustment to age, gender, BMI and echocardiographic predictors of adverse outcome showed that compared with no or mild TR, patients with moderate or severe TR were 10% and 45% more likely to die (95% CI: 1.02–1.18, p=0.009 and 1.34–1.57 p&amp;lt;0.001 respectively). Interaction analysis with adjustment to known predictors of poor survival demonstrated that the association of severe TR with survival was dependent on right ventricle (RV) dysfunction and estimated RV systolic pressure (RVSP) with a more pronounced effect on patients with severe RV dysfunction (HR of 1.38 [1.07–1.80] vs. 1.09 [1.00–1.19], p for interaction = 0.01) and a more pronounced effect on patients with estimated RVSP &amp;lt;40 mmHg (HR of 1.60 [1.21–2.11] vs. 1.14 [1.03–1.25], p for interaction &amp;lt;0.001). Finally, a propensity score matching of patients with severe TR (N=1,154) and matched controls with no or mild TR successfully demonstrated that patients with severe TR were 27% more likely to die during follow up (95% CI: 1.14–1.42, p&amp;lt;0.001). Conclusions Severe TR is independently associated with poor survival. The association is modified by RV dysfunction and estimated RVSP. This report supports the need for studies to evaluate TR interventions on patients' clinical outcomes. Kaplan Meier Survival Curves Funding Acknowledgement Type of funding source: None </jats:sec

Invasive- versus computed tomography-angiography for the evaluation of coronary artery disease among elderly patients undergoing transcatheter aortic valve implantation

Abstract Background Coronary artery disease (CAD) is common among elderly patients with severe aortic stenosis undergoing transcatheter aortic valve implantation (TAVI). Current guidelines recommend that percutaneous coronary intervention (PCI) of &amp;gt;70% proximal coronary lesions prior to TAVI. The aim of the current study was to evaluate two approaches to CAD diagnosis pre-TAVI. Methods We investigated 2,027 patients undergoing TAVI for severe aortic stenosis at two large centers with different pre-procedural CAD assessment: pre-TAVI computed tomography angiography (CTA) with selective invasive angiography according to CTA results (N=831) or mandatory invasive angiography (IA) (N=1,196). Peri-procedural complications were documented according to the VARC-2 criteria. Mortality rates were prospectively documented. Results Mean age of the study population was 86±4, of whom 55% were female. Patients in the IA group had significantly higher rates of pre-TAVI PCI compared to the CTA group (32% vs. 17%, p&amp;lt;0.001). Following TAVI, peri-procedural myocardial infarction (MI) rates were significantly lower among the IA group (0.1% vs. 1.5%, p=0.001). However, multivariate binary logistic regression analysis adjusted for age, gender and cardiovascular risk factors failed to show association between centers strategy and peri-procedural MI. Periprocedural bleeding rates were similar between the groups (3.5% vs. 2.9%, p=0.477). Thirty day, and 1-year mortality crude rates were similar between the groups (2.5% vs. 3.4%, p=0.25, and 10.2% vs. 12.0%, p=0.19). Multivariate cox regression adjusted for age, gender and cardiovascular risk factors did not find association between CAD clearance strategy and outcome. Conclusions In elderly patients, CTA driven approach for CAD evaluation pre-TAVI is a valid strategy with similar outcome as compared to invasive approach. CTA strategy significantly reduces invasive procedures rates without compromising patient's outcome. Funding Acknowledgement Type of funding source: None </jats:sec

Procedural and remote outcome among patients undergoing urgent trans-catheter aortic valve implantation

Abstract Background Severe aortic stenosis patients suffer frequent heart failure decompensations events often requiring hospitalization. In extreme situations patients can be found with pulmonary edema and cardiogenic shock, unresponsive to medical treatment. Urgent trans-catheter aortic valve implantation (TAVI) has emerged as a treatment option for these high-risk patients. Methods We investigated 3,599 patients undergoing TAVI. Subjects were divided into two groups based on procedure urgency: patients who were electively hospitalized for the procedure (N=3,448) and those who had an urgent TAVI (N=151). Peri-procedural complications were documented according to the VARC-2 criteria. In hospital and 1-year mortality rates were prospectively documented. Results Mean age of the study population was 82±7, of whom 52% were female. Peri-procedural complication rates was significantly higher among patients with an urgent indication for TAVI compared to those having an elective procedure: valve malposition 3.6% vs. 0.6% (p-value=0.023), valve migration 3.2% vs. 0.9% (p-value=0.016), post procedure myocardial infarction 3.7% vs. 0.3% (p-value=0.004), and stage 3 acute kidney injury 2.6% vs. 0.5%, (p-value=0.02). Univariate analysis found that patients with urgent indication for TAVI had significantly higher in hospital mortality (5.8% vs. 1.4%, p-value&amp;lt;0.001). similarly, multivariate analysis adjusted for age, gender and cardio-vascular risk factors found that patients with urgent indication had more than 5-folds increased risk of in-hospital mortality (OR 5.94, 95% CI 2.28–15.43, p-value&amp;lt;0.001). Kaplan-Meier's survival analysis showed that patients undergoing urgent TAVI had higher 1-year mortality rates compared to patients undergoing an elective TAVI procedure (p-value log-rank&amp;lt;0.001, Figure). Multivariate analysis found they had more than 2-folds increased risk of mortality at 1-year (HR 2.27, 95% CI 1.53–3.38, p&amp;lt;0.001 compared to those having an elective procedure. Conclusions Patients with urgent indication for TAVI have higher in-hospital mortality and higher peri-procedural complication rates. However, if these patients survive the index hospitalization, they enjoy good prognosis. Kaplan-Meier's survival analysis Funding Acknowledgement Type of funding source: None </jats:sec

2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy.

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Clinical Predictors for Procedural Stroke and Implications for Embolic Protection Devices during TAVR: Results from the Multicenter Transcatheter Aortic Valve Replacement In-Hospital Stroke (TASK) Study

Background: Data to support the routine use of embolic protection devices for stroke prevention during transcatheter aortic valve replacement (TAVR) are controversial. Identifying patients at high risk for peri-procedural cerebrovascular events may facilitate effective patient selection for embolic protection devices during TAVR. Aim: To generate a risk score model for stratifying TAVR patients according to peri-procedural cerebrovascular events risk. Methods and results: A total of 8779 TAVR patients from 12 centers worldwide were included. Peri-procedural cerebrovascular events were defined as an ischemic stroke or a transient ischemic attack occurring ≤24 h from TAVR. The peri-procedural cerebrovascular events rate was 1.4% (n = 127), which was independently associated with 1-year mortality (hazards ratio (HR) 1.78, 95% confidence interval (CI) 1.06-2.98, p &lt; 0.028). The TASK risk score parameters were history of stroke, use of a non-balloon expandable valve, chronic kidney disease, and peripheral vascular disease, and each parameter was assigned one point. Each one-point increment was associated with a significant increase in peri-procedural cerebrovascular events risk (OR 1.96, 95% CI 1.56-2.45, p &lt; 0.001). The TASK score was dichotomized into very-low, low, intermediate, and high (0, 1, 2, 3-4 points, respectively). The high-risk TASK score group (OR 5.4, 95% CI 2.06-14.16, p = 0.001) was associated with a significantly higher risk of peri-procedural cerebrovascular events compared with the low TASK score group. Conclusions: The proposed novel TASK risk score may assist in the pre-procedural risk stratification of TAVR patients for peri-procedural cerebrovascular events

2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy.

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