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Abstract Background Adults with congenital heart defects (ACHD) globally constitute a notably medically underserved patient population. Despite therapeutic advancements, these individuals often confront substantial physical and psychosocial residua or sequelae, requiring specialized, integrative cardiological care throughout their lifespan. Heart failure (HF) is a critical challenge in this population, markedly impacting morbidity and mortality. Aims The primary aim of this study is to establish a comprehensive, prospective registry to enhance understanding and management of HF in ACHD. Named PATHFINDER-CHD, this registry aims to establish foundational data for treatment strategies as well as the development of rehabilitative, prehabilitative, preventive, and health-promoting interventions, ultimately aiming to mitigate the elevated morbidity and mortality rates associated with congenital heart defects (CHD). Methods This multicenter survey will be conducted across various German university facilities with expertise in ACHD. Data collection will encompass real-world treatment scenarios and clinical trajectories in ACHD with manifest HF or at risk for its development, including those undergoing medical or interventional cardiac therapies, cardiac surgery, inclusive of pacemaker or ICD implantation, resynchronization therapy, assist devices, and those on solid organ transplantation. Design The study adopts an observational, exploratory design, prospectively gathering data from participating centers, with a focus on patient management and outcomes. The study is non-confirmatory, aiming to accumulate a broad spectrum of data to inform future hypotheses and studies. Processes Regular follow-ups will be conducted, systematically collecting data during routine clinical visits or hospital admissions, encompassing alterations in therapy or CHD-related complications, with visit schedules tailored to individual clinical needs. Assessments Baseline assessments and regular follow-ups will entail comprehensive assessments of medical history, ongoing treatments, and outcomes, with a focus on HF symptoms, cardiac function, and overall health status. Discussion of the design The design of the PATHFINDER-CHD Registry is tailored to capture a wide range of data, prioritizing real-world HF management in ACHD. Its prospective nature facilitates longitudinal data acquisition, pivotal for comprehending for disease progression and treatment impacts. Conclusion The PATHFINDER-CHD Registry is poised to offer valuable insights into HF management in ACHD, bridging current knowledge gaps, enhancing patient care, and shaping future research endeavors in this domain.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19–58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning–Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning–Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

Abstract Background Pericardial tamponade (PT) early after cardiac surgery is a challenging clinical entity, not infrequently misrecognized and often only detected late in its course. Because the clinical signs of pericardial tamponade can be very unspecific, a high degree of initial suspicion is required to establish the diagnosis. In addition to clinical examination the deployment of imaging techniques is almost always mandatory in order to avoid delays in diagnosis and to initiate any necessary interventions, such as pericardiocentesis or direct cardiac surgical interventions. After a brief overview of how knowledge of PT has developed throughout history, we report on an atypical life-threatening cardiac tamponade after cardiac surgery. A 74-year-old woman was admitted for elective biological aortic valve replacement and aorto-coronary-bypass grafting (left internal mammary artery to left anterior descending artery, single vein graft to right coronary artery). On the 10th postoperative day, the patient unexpectedly deteriorated. She rapidly developed epigastric pain radiating to the left upper abdomen, and features of low peripheral perfusion and shock. There were no clear signs of pericardial tamponade either clinically or echocardiographically. Therefore, for further differential diagnosis, a contrast-enhanced computed tomography scan was performed under clinical suspicion of acute abdomen. Unexpectedly, active bleeding distally from the right coronary anastomosis was revealed. While the patient was prepared for operative revision, she needed cardiopulmonary resuscitation, which was successful. Intraoperatively, the source of bleeding was located and surgically relieved. The subsequent postoperative course was uneventful. Conclusions In the first days after cardiac surgery, the occurrence of life-threatening situations, such as cardiac tamponade, must be expected. Especially if the symptoms are atypical, the entire diagnostic armamentarium must be applied to identify the origin of the complaints, which may be cardiac, but also non-cardiac. Central message A high level of suspicion, immediate diagnostic confirmation, and rapid treatment are required to recognize and successfully treat such an emergency (Fig. 5). Perspective Pericardial tamponade should always be considered as a complication of cardiac surgery, even when symptoms are atypical. The full range of diagnostic tools must be used to identify the origin of the complaints, which may be cardiac, but also non-cardiac (Fig. 5).

Abstract We present time‐correlated ultra‐high‐speed video camera and electromagnetic field measurements of the attachment processes in a natural negative cloud‐to‐ground stroke. The video camera frame exposure time and pixel resolution were 740 ns and 0.91 m/pixel, respectively. The common streamer zone (CSZ) was first observed 2.52 µs preceding the first frame showing the return stroke (RS) in progress, when the upward and downward leader‐tips were 9.8 m apart. In the next frame, the two leaders were observed to have propagated toward each other within the CSZ, with their tips being 0.91 m apart. Our observations show with unprecedented precision/clarity that (a) the slow front in the field waveform is associated with the CSZ, and (b) the “proper” start of the RS is marked by the onset of the fast transition in the field waveform which occurs at the completion of the attachment processes (when the upward and downward leaders have merged).

Malignant cardiac tumors of the heart are extremely rare and may present tremendous diagnostic and therapeutic challenges. These tumors are able to infiltrate the heart and metastasize systemically. Early detection is often elusive as the clinical presentation is highly variable, posing significant diagnostic and therapeutic difficulties. Despite a multidisciplinary approach, the prognosis for patients with malignant cardiac tumors remains guarded. Early diagnosis and a multidisciplinary approach involving cardiac surgeons, oncologists and critical care specialists are crucial in the management of this disease. Further research is needed to better understand the pathomechanisms of tumor-related complications and to develop effective treatment strategies to improve patient outcomes. The rare case of a 78-year-old woman with left atrial tumor requiring emergency surgery for acutely developing mitral valve obstruction is presented. Pathology confirmed an undifferentiated pleomorphic sarcoma. This patient tragically did not survive, highlighting the difficulties of managing such a rare and deceptive heart disease.

Concomitant LAA occlusion has been shown to be an effective and safe treatment for patients with atrial fibrillation during cardiac surgery to prevent embolic stroke. Minimally invasive procedures are challenging due to restricted access to and visibility of the surgical site. Also, aortic endoclamping has been developed as an alternative surgical approach to exoclamping. The aim of this article is to demonstrate the method of beating heart LAA occlusion with the Atriclip® (AtriCure, Mason, OH, USA) device during minimally invasive mitral valve surgery while using the endoclamping alternative for aortic cross-clamping.

Infective endocarditis (IE) is still a life-threatening disease with high morbidity and mortality. While usually caused by a single bacterium, poly-microbial infective endocarditis (IE) is rare. Here, we report a (blood-culture-negative) dual pathogen mitral valve IE caused by Coxiella burnetii and Streptococcus gordonii: A 53-year-old woman was presented to an internal medicine department with abdominal pain for further evaluation. Within the diagnostic work up, transthoracic echocardiography (TTE) revealed an irregularly shaped echogenic mass (5 × 13 mm) adherent to the edge of the posterior mitral valve leaflet and protruding into the left atrium. As infected endocarditis was suspected, blood cultures were initially obtained, but they remained negative. Chronic Q fever infection was diagnosed using serologic testing. After the occurrence of cerebral thromboembolic events, the patient was admitted for mitral valve surgery. Intraoperatively, a massively destructed mitral valve with adhering vegetations was noted. Examination of the mitral valve by broad-range bacterial polymerase chain reaction (PCR) and amplicon sequencing confirmed Coxiella burnetii infection and yielded Streptococcus gordonii as the second pathogen. Based on the detailed diagnosis, appropriate antibiotic therapy of both pathogens was initiated, and the patient could be discharged uneventfully on the 11th postoperative day after a successful minimal-invasive mitral valve replacement.

Abstract The proliferation of disposable, wearable, and implantable printable electronics requires the development of high‐performance biodegradable, and sustainable electronic components. Often green materials don't have the necessary properties for high‐performance electronics, therefore obtaining the ideal properties requires a combination of multiple green materials. A tri‐layer dielectric is reported using poly(lactic acid) (PLA), poly(vinyl alcohol)/cellulose nanocrystals (PVAc), and toluene diisocyanate terminated poly(caprolactone) (TPCL), which is integrated into semiconducting single‐walled carbon nanotube (sc‐SWCNT) based thin film transistors (TFTs) in a top gate bottom contact architecture. The PVA provides a high dielectric constant due to the hydroxy groups, the cellulose is used to optimize the viscosity, the TPCL layer provides a robust hydrophobic surface, and the PLA eliminates the interfacial charge traps present in the PVAc and improves the adhesion between PVAc and the substrate. This leads to a decrease in leakage currents and reduces the polarity at the dielectric/semiconductor interface. The TFTs fabricated using tri‐layer dielectrics led to air‐stable n‐type devices with higher overall performance when compared against the PVAc/TPCL bilayer devices.

(1) Background: Systemic mastocytosis is a rare, non-curable disease with potential life-threatening complications in patients receiving cardiac surgery. (2) Methods: This systematic review of the literature was prompted by the case of a life-threatening anaphylactic reaction during cardiac surgery related to systemic mastocytosis. The search of all types of studies, using several databases (Pubmed, Scopus and Web of Science), was conducted through September 2022 to identify the relevant studies. (3) Results: Twelve studies were included describing cases of patients undergoing cardiac surgery who were diagnosed with systemic mastocytosis. An adverse effect, namely anaphylaxis, has happened in three cases. Different strategies of premedication, intraoperative and postoperative management were used. In our case, the patient was admitted for elective biological aortic valve replacement due to severe aortic stenosis. Intraoperatively, the patient developed an anaphylactic shock during the administration of protamine after separation from the cardiopulmonary bypass. This anaphylaxis reaction was a complication of the pre-existing systemic mastocytosis and could be successfully managed by the administration of epinephrine, antihistamines and corticosteroids. (4) Conclusions: This systematic literature search and case report highlight the importance of careful preoperative planning, as well as coordination between cardiac surgeons, anesthesiologists and hemato-oncological specialists, in patients with rare but complication-prone diseases such as systemic mastocytosis.

Abstract Background Peroxisome proliferator activated receptor gamma (PPARG) belongs to the nuclear receptor superfamily functioning as transcription factors to regulate cellular differentiation, development and metabolism. Moreover, it has been implicated in the regulation of lipid metabolism, as well as the maturation of monocytes/macrophages and the control of inflammatory reactions. The aim of this study was to evaluate the relationship between the Pro12Ala (rs1808212) PPARG gene polymorphism on immune molecular and cellular components in mothers and their offspring participating in the PREOBE study. Methods DNA from maternal venous blood samples at 24, 34 and 40 gestational weeks, plus cord blood samples was extracted. Pro12Ala PPARG polymorphism genotyping was performed, and immune system markers were analyzed by flow cytometry. Results Study findings revealed no effect of rs1808212 PPARG genotypes on innate immune parameters in mothers and their offspring; however, CD4 + /CD8 + ratio were decreased at 24 and 34 weeks in pregnant women carrying the CG (Pro12Ala) rs1808212 polymorphism, (p = 0,012 and p = 0,030; respectively). Only CD19 levels in peripheral blood were significantly higher at delivery in pregnant women carrying the CC (Pro12Pro) genotype (p ≤ 0.001). Moreover, there were statistically significant differences in leukocytes and neutrophils maternal levels at 34 weeks of gestation, being lower in carriers of Pro12Ala genotype (p = 0.028 and p = 0.031, respectively). Conclusions Results suggest that Pro12Ala PPARG polymorphism may have an effect on some cell and immune parameters in pregnant women during pregnancy and at time of delivery. However, newborn innate immune system does not seems to be influenced by PPARG Pro12Ala polymorphism in cord blood.

Abstract Data-driven machine learning (ML) has emerged as a promising approach for building accurate and robust statistical models from medical data, which is collected in huge volumes by modern healthcare systems. Existing medical data is not fully exploited by ML primarily because it sits in data silos and privacy concerns restrict access to this data. However, without access to sufficient data, ML will be prevented from reaching its full potential and, ultimately, from making the transition from research to clinical practice. This paper considers key factors contributing to this issue, explores how federated learning (FL) may provide a solution for the future of digital health and highlights the challenges and considerations that need to be addressed.

Background: It is clinically widely overlooked that many patients with Marfan- (MFS) or Loeys-Dietz-Syndrome (LDS) are obese. While anthropometric routine parameters are not very suitable, the modern Bioelectrical Impedance Analysis (BIA) seems superior for the acquisition of reliable noninvasive assessment of body composition of patients. The aim of the study was to assess the body composition of patients with MFS/LDS by BIA in order to detect occult obesity, which may be a risk marker for aortic or vascular complications. Methods: In this exploratory cross-sectional study, 50 patients (66% female; mean age: 37.7 ± 11.7 [range: 17–64] years) with a molecular genetic (n = 45; 90%) or clinical (n = 5; 10%) proven diagnosis of MFS or LDS were enrolled between June 2020 and February 2022. All BIA-measurements were performed with the Multifrequence-Impedance-Analyzer Nutriguard-MS (Data Input, Poecking, Germany). Results: The MFS/LDS collective was significantly different from an age-, sex-, and BMI-adjusted control in terms of body fat, percent cellularity, body cell mass, extra cellular mass/body cell mass index, and phase angle (all p < 0.05). The mean BIA-measured bodyfat was 31.7 ± 8.7% [range: 9.5–53.5%], while the mean calculated BMI of the included patients was 23.0 ± 4.8 kg/m2 [range: 15.2–41.9 kg/m2]. Therefore, using the obesity cut-off values for the body fat percentage of 25% in men and 35% in women, the BIA classifies as many as 28 patients (56.0%) as obese. In contrast only 12 patients (24.0%) were pre-obese, respectively 3 (6.0%) obese by BMI. The significant difference (p < 0.001) had an accordance of 42.7%. Overall, 15 patients (13 MFS; 2 LDS) had previous aortic surgery (n = 14) and/or interventional treatment (n = 2) for aortic complications (aneurysm, aortic dissection). 11 out of these 15 (73.3%) were currently classified as obese by BIA. Conclusions: The fact that many patients with MFS or LDS are obese is widely unknown, although obesity may be associated with impaired vascular endothelial function and an increased risk of cardiovascular complications. Also, in patients with MFS/LDS, BIA allows a reliable assessment of the body composition beyond the normal anthropometric parameters, such as BMI. In the future, BIA-data possibly may be of particular importance for the assessment of the vascular risk of MFS/LDS patients, besides the aortic diameters.

Abstract Managing spatially structured populations of imperiled species presents many challenges. Spatial structure can make it difficult to predict population responses to potential recovery activities, and learning through experimentation may not be advised if it could harm threatened populations. Adaptive management provides an appealing framework when experimentation is considered too risky or time consuming; we used such an approach for imperiled flatwoods salamanders at a Florida wildlife refuge. We represented this metapopulation with category count models and used stochastic dynamic programming to identify optimal decision policies that weighed trade‐offs between metapopulation persistence and management costs. We defined possible wetland categories in terms of habitat suitability and occupancy, specified category‐specific management actions, and identified transition probabilities via expert elicitation for two management strategies: “future” status quo (FSQ; frequent growing‐season burns) and extra management actions (EMA; restoration, translocation, head‐starting). We simulated metapopulation dynamics using the resulting optimal management policy and found that under model FSQ, occupancy steadily declined over time, indicating that populations would rapidly become extirpated; with model EMA, occupancy remained stable, suggesting that populations would persist only if additional actions are applied and are effective. This approach can be used to identify optimal solutions while accounting for uncertainty and considering both habitat and population dynamics, and to help managers make conservation decisions for populations at imminent risk of extinction.

This study describes the development of a prototype bi-spectral microbolometer sensor system designed explicitly for radiometric measurement and characterization of wildfire mid- and long-wave infrared radiances. The system is tested experimentally over moderate-scale experimental burns coincident with FLIR reference imagery. Statistical comparison of the fire radiative power (FRP; W) retrievals suggest that this novel system is highly reliable for use in collecting radiometric measurements of biomass burning. As such, this study provides clear experimental evidence that mid-wave infrared microbolometers are capable of collecting FRP measurements. Furthermore, given the low resource nature of this detector type, it presents a suitable option for monitoring wildfire behaviour from low resource platforms such as unmanned aerial vehicles (UAVs) or nanosats.

Complex oxides: Simulating the complete phase diagram of nickelates A new theoretical approach provides a complete phase diagram of rare-earth nickelates, reproducing the key features seen in experiments. Transition metal oxides with a pervoskite crystal structure exhibit a broad range of behaviours due to a complex the interplay between lattice, electronic and magnetic degrees of freedom. Rare-earth nickelates are a particularly interesting class of perovskite oxide that undergo a highly tunable (and potentially exploitable) metal-insulator transition. Theoretically describing the insulating phase of nickelates, however, is far from trivial and two seemingly distinct descriptions have emerged. A team of researchers led by Manuel Bibes at Unité Mixte de Physique CNRS/Thales use first-principles simulations to somewhat reconcile these conflicting visions, fully describing the electronic and magnetic ground state of nickelates, as well as hinting at strategies for tuning these fascinating materials.

PurposeThe study objective was to compare the prognostic value of interim and end-of-treatment FDG PET/CT using five therapeutic evaluation criteria in patients with diffuse large B cell lymphoma (DLBCL).Methods181 patients were retrospectively analysed. All patients underwent FDG-PET at baseline and after four cycles (iPET4) of first-line chemotherapy and 165 at the end-of-treatment (PET-eot). Ratio Deauville score (rDS) (SUVmax-target residual lesion/SUVmax-liver) was measured in iPET4 and PET-eot, and its optimal threshold was determined using receiver operating characteristic (ROC) curve analysis. Deauville score (DS) (iPET4 and PET-eot), ΔSUVmax, ΔSUVmax determined according to Menton 2011 criteria (ΔSUVmax+DS) and ΔSUVmax+rDS were also evaluated (iPET4 only). Median follow-up was 44 months.ResultsROC analysis revealed the optimal cut-off value was 1.4-fold of SUVmax-liver on iPET4 and PET-eot. On iPET4, positive predictive value (PPV) of rDS was significantly better than DS: 81.58% vs. 67.79%. In univariate analysis, the five interpretation methods were statistically significant (pConclusionsrDS with a tumor/liver ratio of 1.4 is a robust prognostic factor in patients with DLBCL on iPET4 and PET-eot.

A library of statistically random pentafluorostyrene (PFS) and methyl methacrylate (MMA) copolymers with narrow molecular weight distributions was produced, using nitroxide mediated polymerization (NMP) to study the effect of polymer composition on the performance of bottom-gate top-contact organic thin-film transistors, when utilized as the dielectric medium. Contact angle measurements confirmed the ability to tune the surface properties of copolymer thin films through variation of its PFS/MMA composition, while impedance spectroscopy determined the effect of this variation on dielectric properties. Bottom-gate, top-contact copper phthalocyanine (CuPc) based organic thin-film transistors were fabricated using the random copolymers as a dielectric layer. We found that increasing the PFS content led to increased field-effect mobility, until a point after which the CuPc no longer adhered to the polymer dielectric.

Recent research has shown that auditory closed-loop stimulation can enhance sleep slow oscillations (SO) to improve N3 sleep quality and cognition. Previous studies have been conducted in lab environments. The present study aimed to validate and assess the performance of a novel ambulatory wireless dry-EEG device (WDD), for auditory closed-loop stimulation of SO during N3 sleep at home. The performance of the WDD to detect N3 sleep automatically and to send auditory closed-loop stimulation on SO were tested on 20 young healthy subjects who slept with both the WDD and a miniaturized polysomnography (part 1) in both stimulated and sham nights within a double blind, randomized and crossover design. The effects of auditory closed-loop stimulation on delta power increase were assessed after one and 10 nights of stimulation on an observational pilot study in the home environment including 90 middle-aged subjects (part 2).The first part, aimed at assessing the quality of the WDD as compared to a polysomnograph, showed that the sensitivity and specificity to automatically detect N3 sleep in real-time were 0.70 and 0.90, respectively. The stimulation accuracy of the SO ascending-phase targeting was 45 ± 52°. The second part of the study, conducted in the home environment, showed that the stimulation protocol induced an increase of 43.9% of delta power in the 4 s window following the first stimulation (including evoked potentials and SO entrainment effect). The increase of SO response to auditory stimulation remained at the same level after 10 consecutive nights. The WDD shows good performances to automatically detect in real-time N3 sleep and to send auditory closed-loop stimulation on SO accurately. These stimulation increased the SO amplitude during N3 sleep without any adaptation effect after 10 consecutive nights. This tool provides new perspectives to figure out novel sleep EEG biomarkers in longitudinal studies and can be interesting to conduct broad studies on the effects of auditory stimulation during sleep.

Deriving tractable reduced equations of biological neural networks capturing the macroscopic dynamics of sub-populations of neurons has been a longstanding problem in computational neuroscience. In this paper, we propose a reduction of large-scale multi-population stochastic networks based on the mean-field theory. We derive, for a wide class of spiking neuron models, a system of differential equations of the type of the usual Wilson-Cowan systems describing the macroscopic activity of populations, under the assumption that synaptic integration is linear with random coefficients. Our reduction involves one unknown function, the effective non-linearity of the network of populations, which can be analytically determined in simple cases, and numerically computed in general. This function depends on the underlying properties of the cells, and in particular the noise level. Appropriate parameters and functions involved in the reduction are given for different models of neurons: McKean, Fitzhugh-Nagumo and Hodgkin-Huxley models. Simulations of the reduced model show a precise agreement with the macroscopic dynamics of the networks for the first two models.