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Highly magnified micrographs are part of the majority of publications in materials science and related fields. They are often the basis for discussions and far-reaching conclusions on the nature of the specimen. In many cases, reviewers demand and researchers deliver only the bare minimum of micrographs to substantiate the research hypothesis at hand. In this work, we use heterogeneous poly(acrylonitrile) nanofiber nonwovens with embedded nanoparticles to demonstrate how an insufficient or biased micrograph selection may lead to erroneous conclusions. Different micrographs taken by transmission electron microscopy and helium ion microscopy with sometimes contradictory implications were analyzed and used as a basis for micromagnetic simulations. With this, we try to raise awareness for the possible consequences of cherry-picking for the reliability of scientific literature.

Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive and fatal condition that requires early diagnosis, management, and specific treatment. The availability of new disease-modifying therapies has made successful treatment a reality. Transthyretin amyloid cardiomyopathy can be either age-related (wild-type form) or caused by mutations in the TTR gene (genetic, hereditary forms). It is a systemic disease, and while the genetic forms may exhibit a variety of symptoms, a predominant cardiac phenotype is often present. This document aims to provide an overview of ATTR-CM amyloidosis focusing on cardiac involvement, which is the most critical factor for prognosis. It will discuss the available tools for early diagnosis and patient management, given that specific treatments are more effective in the early stages of the disease, and will highlight the importance of a multidisciplinary approach and of specialized amyloidosis centres. To accomplish these goals, the World Heart Federation assembled a panel of 18 expert clinicians specialized in TTR amyloidosis from 13 countries, along with a representative from the Amyloidosis Alliance, a patient advocacy group. This document is based on a review of published literature, expert opinions, registries data, patients' perspectives, treatment options, and ongoing developments, as well as the progress made possible via the existence of centres of excellence. From the patients' perspective, increasing disease awareness is crucial to achieving an early and accurate diagnosis. Patients also seek to receive care at specialized amyloidosis centres and be fully informed about their treatment and prognosis.

Most patients admitted for acute heart failure have normal or increase blood pressure. Relaxin is a natural human peptide that affects multiple vascular control pathways, suggesting potential mechanisms of benefit for such patients. We assessed the dose response of relaxin's effect on symptom relief, other clinical outcomes, and safety. In a placebo-controlled, parallel-group, dose-ranging study, 234 patients with acute heart failure, dyspnoea, congestion on chest radiograph, and increased brain natriuretic peptide (BNP) or N-terminal prohormone of BNP, mild-to-moderate renal insufficiency, and systolic blood pressure greater than 125 mm Hg were recruited from 54 sites in eight countries and enrolled within 16 h of presentation. Patients were randomly assigned, in a double-blind manner via a telephone-based interactive voice response system, to standard care plus 48-h intravenous infusion of placebo (n=62) or relaxin 10 μg/kg (n=40), 30 μg/kg (n=43), 100 μg/kg (n=39), or 250 μg/kg (n=50) per day. Several clinical endpoints were explored to assess whether intravenous relaxin should be pursued in larger studies of acute heart failure, to identify an optimum dose, and to help to assess endpoint selection and power calculations. Analysis was by modified intention to treat. This study is registered with ClinicalTrials.gov, number NCT00520806. In the modified intention-to-treat population, 61 patients were assessed in the placebo group, 40 in the relaxin 10 μg/kg per day group, 42 in the relaxin 30 μg/kg per day group, 37 in the relaxin 100 μg/kg per day group, and 49 in the relaxin 250 μg/kg per day group. Dyspnoea improved with relaxin 30 μg/kg compared with placebo, as assessed by Likert scale (17 of 42 patients [40%] moderately or markedly improved at 6 h, 12 h, and 24 h vs 14 of 61 [23%]; p=0·044) and visual analogue scale through day 14 (8214 mm×h [SD 8712] vs 4622 mm×h [9003]; p=0·053). Length of stay was 10·2 days (SD 6·1) for relaxin-treated patients versus 12·0 days (7·3) for those given placebo, and days alive out of hospital were 47·9 (10·1) versus 44·2 (14·2). Cardiovascular death or readmission due to heart or renal failure at day 60 was reduced with relaxin (2·6% [95% CI 0·4–16·8] vs 17·2% [9·6–29·6]; p=0·053). The number of serious adverse events was similar between groups. When given to patients with acute heart failure and normal-to-increased blood pressure, relaxin was associated with favourable relief of dyspnoea and other clinical outcomes, with acceptable safety. Corthera (USA).

In hypertrophic cardiomyopathy (HCM) patients, left ventricular (LV) maximal wall thickness (MWT) is one of the most important factors determining sudden cardiac death (SCD) risk. In a large unselected sample of HCM patients, we aimed to simulate what changes would occur in the calculated SCD risk according to the European HCM Risk-SCD calculator when MWT measured using echocardiography was changed to MWT measured using MRI. All consecutive patients with HCM who underwent cardiac MRI were included. MWT measured with echocardiography and MRI were compared, and 5-year SCD risk according to the HCM Risk-SCD calculator was computed using four different models. The final population included 673 patients [389 (57.8%) males, median age 50 years, interquartile range (36–60)]. The median MWT was lower measured by echocardiography than by MRI [20 (17–24) mm vs 21 (18–24) mm; p  < 0.0001]. There was agreement between echocardiography and MRI in the measurement of maximal LV wall thickness in 96 patients (14.3%). The largest differences between echo and MRI were − 13 mm and + 9 mm. The differences in MWT by echocardiography and MRI translated to a maximal difference of 8.33% in the absolute 5-year risk of SCD , i.e., the echocardiography-based risk was 8.33% lower than the MRI-based estimates. Interestingly, 13.7% of patients would have been reclassified into different SCD risk categories if MRI had been used to measure MWT instead of echocardiography. In conclusion, although there was high general intermodality agreement between echocardiography and MRI in the MWT measurements, the differences in MWT translated to significant differences in the 5-year risk of SCD.

TTN gene truncating variants are common in dilated cardiomyopathy (DCM), although data on their clinical significance is still limited. We sought to examine the frequency of truncating variants in TTN in patients with DCM, including familial DCM (FDCM), and to look for genotype-phenotype correlations. Clinical cardiovascular data, family histories and blood samples were collected from 72 DCM probands, mean age of 34 years, 45.8% FDCM. DNA samples were examined by next generation sequencing (NGS) with a focus on the TTN gene. Truncating mutations were followed up by segregation study among family members. We identified 16 TTN truncating variants (TTN trunc) in 17 probands (23.6% of all cases, 30.3% of FDCM, 17.9% of sporadic DCM). During mean 63 months from diagnosis, there was no difference in adverse cardiac events between probands with and without TTN truncating mutations. Among relatives 29 mutation carriers were identified, nine were definitely affected (31%), eight probably affected (27.6%) one possibly affected (3.4%) and eleven were not affected (37.9%). When relatives with all affected statuses were combined, disease penetrance was still incomplete (62.1%) even after exclusion of unaffected relatives under 40 (82%) and was higher in males versus females. In all mutation carriers, during follow-up, 17.4% had major adverse cardiac events, and prognosis was significantly worse in men than in women. In conclusion, TTN truncating variants were observed in nearly one fourth of young DCM patient population, in vast majority without conduction system disease. Incomplete penetrance suggests possible influence of other genetic and/or environmental factors on the course of cardiotitinopathy. Counseling should take into account sex and incomplete penetrance.

Objectives To assess the value of cardiac MRI in comparison to echocardiography in consecutive patients with previously diagnosed and new suspected hypertrophic cardiomyopathy (HCM). Methods All MRI studies of patients with HCM or suspected disease performed at our centre within a 10-year time period were evaluated. Initial diagnoses (echocardiography-based) and final (MRI-based) diagnoses were compared in subgroups, and the discrepancies were recorded. Results A total of 1006 subjects with HCM or suspected HCM were identified (61% males, 39% females; median age, 49.1 years; interquartile range, 34.9–60.4). In 12 (2.2%) out of 550 patients with known HCM, MRI indicated a diagnosis other than HCM, including but not limited to the subaortic membrane ( n  = 1, 8.3%) or mild left ventricular hypertrophy ( n  = 5, 41.7%). Among all patients with suspected HCM ( n  = 456), MRI diagnosis was different from HCM in 5.3% ( n  = 24) of patients. In an additional 20.4% of patients ( n  = 93), no significant hypertrophy was present. In total, among patients with suspected HCM, MRI led to clear HCM diagnosis in 204 (44.7%) patients. Among patients with a history of uncontrolled hypertension suspected of having HCM, MRI aided in identifying cardiomyopathy in 47.9% of patients. This subgroup contained the largest proportion of patients with an ambiguous diagnosis, namely, 29.6% compared with 13.8% in the remaining groups of patients with suspected HCM ( p  = 0.0001). Conclusions In a small but important group of patients with ultrasound-based HCM, cardiac MRI can diagnose previously unknown conditions and/or refute suspected cardiomyopathy. The diagnostic yield of MRI when compared to echocardiography in patients suspected of having HCM is 44.7%. Key Points • Out of 550 patients previously diagnosed with echocardiography but without magnetic resonance imaging (MRI) as having hypertrophic cardiomyopathy (HCM), we diagnosed a different disease in 12 (2.2%) patients using MRI. • Among patients with suspected HCM based on echocardiography, MRI led to clear HCM diagnosis in 44.7% of patients. • In patients with a history of uncontrolled hypertension suspected, based on an echocardiogram, of having HCM, MRI aided in identifying cardiomyopathy in 47.9% of patients. This subgroup contained the largest proportion of patients with an ambiguous diagnosis.

Background/Objectives: Having serum biomarkers available for cardiac transthyretin amyloidosis (ATTR-CA) would be beneficial for diagnosis and prognosis. This study aimed to identify potential ATTR-CA biomarkers through proteomic analysis. Patients and Methods: Serum proteomic analyses were conducted on 15 ATTR-CA patients before receiving treatment, 11 ATTR-CA patients who had received tafamidis treatment for at least six months, and 13 patients with suspected cardiac amyloidosis who were later ruled out. All patients underwent blood tests, standard 12-lead electrocardiography, transthoracic echocardiography, and 99mTc-DPD scintigraphy. Results: Proteomic analysis revealed significant differences in protein levels among the study groups. Key findings revealed increased levels of several proteins, including ceruloplasmin, apolipoprotein E, SERPINA1, and cDNA FLJ54111 (which is highly similar to serum transferrin), in ATTR-CA patients before receiving specific treatment. There was also a reduction in prothrombin, transferrin, CD14, and alpha-2-macroglobulin. In the ATTR-CA group treated with tafamidis, elevated levels of SERPINA1, paraoxonase 1, and complement C2 were observed. Notably, levels of cDNA FLJ54111 and SERPINA3 were reduced in this group. Compared to the control group, patients with ATTR-CA exhibited higher levels of ceruloplasmin, SERPINA3, and VCAM1, as well as lower levels of ApoA-I, ApoA-II, clusterin, and gelsolin. Controls exhibited elevated levels of transthyretin and prothrombin. Conclusions: This study identified candidate serum biomarkers for diagnosing ATTR-CA and monitoring the effectiveness of tafamidis treatment.

Background/Objectives: Several predictive models have been proposed to estimate the probability of cardiac transthyretin amyloidosis (ATTR-CA). The aim of our study was to evaluate the usefulness of electrocardiographic parameters, as well as parameters consisting of a combination of myocardial thickness and QRS voltage, as potential predictors of ATTR-CA. Methods: In 2018–2025, 285 consecutive patients with suspected cardiac amyloidosis were evaluated, including blood tests, standard 12-lead electrocardiography, transthoracic echocardiography, and [99mTc]Tc-DPD scintigraphy. Results: The ratio of posterior wall thickness to minimum QRS voltage in limb leads (PWT/minQRS ratio) as well as several ECG-derived parameters were independent predictors of ATTR-CA. In a comparison of ROC curves, PWT/minQRS ratio exceeded both the minimum and maximum voltage of QRS complexes in limb leads, demonstrated similar predictive value to TCAS and T-amylo scores, and had similar or superior predictive characteristics to posterior wall thickness. A cut-off of >3.3 for PWT/minQRS ratio was as accurate as the published cut-offs for TCAS score ≥6, T-amylo score ≥7, and posterior wall thickness ≥14 mm. In the subgroup of patients with myocardial thickness of at least 15 mm, PWT/minQRS ratio >3.3 was superior to posterior wall thickness ≥14 mm and T-amylo score ≥7 and had similar predictive value for ATTR-CA as TCAS score ≥6. Conclusions: In a cohort of undifferentiated patients referred for [99mTc]Tc-DPD scintigraphy due to suspected cardiac amyloidosis, PWT/minQRS ratio showed strong predictive value for ATTR-CA, which was even more pronounced in the subgroup of patients with increased myocardial thickness.

Transthyretin amyloidosis (ATTR) is a rare, life-threatening systemic disorder. We present first findings on the cardiac hereditary ATTR in Poland. Sixty-eight consecutive patients with suspected or known cardiac amyloidosis were evaluated, including blood tests, standard 12-lead electrocardiography (ECG) and transthoracic echocardiography. ATTR was confirmed histologically or non-invasively using 99mTc-DPD scintigraphy. Transthyretin (TTR) gene sequencing was performed. In 2017-2019, 10 unrelated male patients were diagnosed with hereditary ATTR. All patients had very uncommon TTR gene mutations: 7 patients had p.Phe53Leu mutation, 2 patients had p.Glu109Lys mutation and 1 patient had p.Ala101Val mutation. The age of onset ranged from 49 to 67 years (mean [SD] age, 58.7 [6.4] years). On ECG, most patients (70%) had pseudoinfarct pattern and/or low QRS voltage. The maximal wall thickness (MWT) on echocardiography varied considerably among the patients from moderate (16 mm) to massively increased (30 mm). Most patients (90%) had decreased left ventricular ejection fraction (mean [SD], 43 [11] %). On follow-up, we observed progressive heart failure in almost all cases. The first patient with p.Phe53Leu mutation died of heart failure, the second died suddenly, the third successfully underwent combined heart and liver transplant with 15 months survival from the surgery. The patient with p.Ala101Val mutation died of stroke. According to available data, this is the first time that the types of TTR mutations and the clinical characteristics of Polish patients with cardiac hereditary ATTR have been described. Previous literature data about Polish background in families with p.Phe53Leu mutation and the present results, suggest that this TTR mutation might be endemic in the Polish population.

Computers nowadays have different components for data storage and data processing, making data transfer between these units a bottleneck for computing speed. Therefore, so-called cognitive (or neuromorphic) computing approaches try combining both these tasks, as is done in the human brain, to make computing faster and less energy-consuming. One possible method to prepare new hardware solutions for neuromorphic computing is given by nanofiber networks as they can be prepared by diverse methods, from lithography to electrospinning. Here, we show results of micromagnetic simulations of three coupled semicircle fibers in which domain walls are excited by rotating magnetic fields (inputs), leading to different output signals that can be used for stochastic data processing, mimicking biological synaptic activity and thus being suitable as artificial synapses in artificial neural networks.