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Supraventricular tachyarrhythmias pose a significant challenge in neonates and infants, particularly within the first year of life, where prompt and effective management is crucial. By synthesizing available evidence and clinical experience, this review aims to provide a comprehensive overview of antiarrhythmic therapy in this vulnerable population, with a focus on narrow QRS supraventricular tachyarrhythmias. This review examines the current understanding of supraventricular tachyarrhythmia management and discusses the challenges associated with antiarrhythmic therapy in newborns and infants during the critical first year of life, evaluating the efficacy and safety of various antiarrhythmic agents commonly utilized in this population, including dosing considerations, adverse effects, and strategies for acute management and prophylactic long-term antiarrhythmic treatment.

Interest in advanced echocardiographic imaging methods is growing. Left atrial strain (LAS) is among recently developed echocardiographic parameters. LAS represents an index of tissue deformation of the left atrium (LA). This parameter is an expression of LA function. Several arrhythmias are caused by impaired LA function. LAS can be assessed with a resting echocardiogram. The evaluation of LAS during stress echocardiography represents another model for assessing LA function. The development of altered LAS during physical or pharmacological stress is a predictor of early LA disease. Our review aims to evaluate the relationship between alterations in LAS and the development of atrial fibrillation (AF), and the diagnostic and prognostic roles of the stress echocardiogram in clinical practice.

Despite great progress in treating atrial fibrillation (AF), especially with the development of increasingly effective invasive techniques for AF ablation, many unanswered questions remain regarding the pathogenic mechanism of the arrhythmia and its prevention methods. The development of AF is based on anatomical and functional alterations in the cardiomyocyte resulting from altered ionic fluxes and cardiomyocyte electrophysiology. Electric instability and electrical remodeling underlying the arrhythmia may result from oxidative stress, also caused by possible mitochondrial dysfunction. The role of mitochondrial dysfunction in the pathogenesis of AF is not yet fully elucidated; however, the reduction in AF burden after therapeutic interventions that improve mitochondrial fitness tends to support this concept. This selected review aims to summarize the mechanisms of mitochondrial dysfunction related to AF and the current pharmacological treatment options that target mitochondria to prevent or improve the outcome of AF.

Background: Cardiac arrest (CA) is a life-critical condition. Patients who survive after CA go into a defined post-cardiac arrest syndrome (PCAS). In this clinical context, the role of the echocardiogram in recent years has become increasingly important to assess the causes of arrest, the prognosis, and any direct and indirect complications dependent on cardiopulmonary resuscitation (CPR) maneu-vers. Methods: We have conduct a narrative revision of literature. Results: The aim of our review is to evaluate the increasingly important role of the transthoracic and transesophageal echocardiogram in the CA phase and especially post-arrest, analyzing the data already present in the literature. Conclusion: Transthoracic and transesophageal echocardiogram in the CA phase take on important diagnostic and prognostic role.

Abstract Background and Aims Cholangiocarcinoma (CCA), a rare and aggressive hepatobiliary malignancy, presents significant clinical management challenges. Despite rising incidence and evolving treatment options, prognosis remains poor, motivating the exploration of real‐world data for enhanced understanding and patient care. Methods This multicenter study analyzed data from 120 metastatic CCA patients at three institutions from 2016 to 2023. Kaplan–Meier curves assessed overall survival (OS), while univariate and multivariate analyses evaluated links between clinical variables (age, gender, tumor site, metastatic burden, ECOG performance status, response to first‐line chemotherapy) and OS. Genetic profiling was conducted selectively. Results Enrolled patients had a median age of 68.5 years, with intrahepatic tumors predominant in 79 cases (65.8%). Among 85 patients treated with first‐line chemotherapy, cisplatin and gemcitabine (41.1%) was the most common regimen. Notably, one‐third received no systemic treatment. After a median 14‐month follow‐up, 81 CCA‐related deaths occurred, with a median survival of 13.1 months. Two clinical variables independently predicted survival: response to first‐line chemotherapy (disease control vs. no disease control; HR: 0.27; 95% CI: 0.14–0.50; p 1 site vs. 1 site; HR: 1.99; 95% CI: 1.04–3.80; p = 0.0366). The three most common genetic alterations involved the ARID1A, tp53, and CDKN2A genes. Conclusions Advanced CCA displays aggressive clinical behavior, emphasizing the need for treatments beyond chemotherapy. Genetic diversity supports potential personalized therapies. Collaborative research and deeper CCA biology understanding are crucial to enhance patient outcomes in this challenging malignancy.

Abstract This paper presents an effective and novel design procedure employing a novelty named as Perturbation Factor to predict the resonance frequency of resonant cavities in SIW technology perturbed by a shape perturbation. The design procedure can be applied in tunable cavities and bandpass filters with shunt reactance of symmetrical inductive window, and regarding the application technologies, rectangular waveguide resonators and resonant cavities in SIW can be employed due to the equivalence of operation between both technologies. Its straightforward application allows a frequency variation of up to 20% using only one metal post which allows the reduction of the fabrication cost and the preservation of the frequency response across the considered bandwidth. To validate the design procedure, two resonant cavities were fabricated, a rectangular resonant cavity designed to operate at 5.0 GHz and 6.0 GHz and a square resonant cavity designed to operate at 6.0 GHz and 7.2 GHz, and frequency variations of 17.74% and 21.81% were obtained for the rectangular and square cavities, respectively. Still regarding the reached results, was verified a mean error of 1.75% for the predicted resonance frequency, which validates the design procedure. The experimental results are in good agreement with the full-wave computational results and electromagnetic theory.

Background: Wearable cardioverter-defibrillators (WCDs) are currently used in patients at temporarily heightened risk for sudden cardiac death (SCD) who are temporarily unable to receive an implantable cardioverter-defibrillator (ICD). WCD can safely record and terminate life-threatening arrhythmias through a non-invasive electrode-based system. The current clinical indications for WCD use are varied and keep evolving as experience with this technology increases. Methods: We reviewed and explored the data behind indications for WCD use and discuss its usefulness in congenital heart disease (CHD) patients. Results: We considered 8 consecutive patients (mean age 35.25 years, range 18–51 years, average duration of WCD use 4 months, range 3–6 months) with complex CHD, in which a WCD was used between June 2018 and January 2022. No sustained ventricular arrhythmias requiring shocks were recorded in the observation period. No inappropriate shocks were recorded. All the patients showed a good compliance and a very high mean wear time per day (21.2 ± 1 h a day). Four patients implanted a permanent device (3 CRT-D, 1 ICD), three underwent cardiac surgery at the end of the WCD period and one is still on the waiting list for the operation. Conclusions: Larger trial could confirm the possible conceivable benefit from an extended use of the WCD in certain populations with complex CHD as in our case series, especially in patients with life-treating ventricular arrhythmias waiting for surgery for residual cardiac defects or in the early phases following the surgical/hemodynamic interventions, patients with tachycardiomyopathy expected to improve after the arrhythmias are removed and patients awaiting implantation of an ICD at high risk due to active infection.

Background/Objectives: Angiotensin receptor neprilysin inhibitor (ARNI) and sodium-glucose co-transporter 2 inhibitors (SGLT2i) are essential medications in heart failure (HF) therapy, and their potential antiarrhythmic effects have been reported. Recently, ARNI and SGLT2i use for HF in adult congenital heart disease (ACHD) has been studied. However, whether any beneficial effects may be achieved on the arrhythmic burden in the complex population of ACHD with a systemic right ventricle (sRV) is still to be determined. Methods: We retrospectively collected all significant arrhythmic events from a cohort of patients with a failing sRV attending our tertiary care center on optimal guideline-directed medical therapy (GDMT) with ARNI and/or SGLT2i. Results: A total of 46 patients (mean age 38.2 ± 10.7 years, 58% male) on sacubitril/valsartan were included. Twenty-three (50%) patients were also started on dapagliflozin. After a median follow-up of 36 [Q1–Q3: 34–38] months, arrhythmic events occurred globally in 13 (28%) patients. Survival analysis showed significant reduction of clinically relevant atrial and ventricular arrhythmia at follow-up (p = 0.027). Conclusions: Our findings suggest that GDMT including sacubitril/valsartan and dapagliflozin may also offer an antiarrhythmic effect in ACHD patients with a failing sRV, by reducing the incidence of arrhythmic events at follow-up. [ABSTRACT FROM AUTHOR]

During embryonic development, muscle tissues, skin, and a subset of vascular endothelial cells arise from Pax3-expressing embryonic progenitors defined as paraxial mesoderm. By contrast, haemogenic potential is well established for extra-embryonic mesoderm and intraembryonic lateral plate mesoderm, which do not express Pax3. To date, it is not known whether the haematopoietic system also contains Pax3 lineage cells. Here, we show that the mouse foetal liver and foetal circulation contain a transient population of Pax3 lineage cells with hallmarks of haematopoietic progenitors and the potential to generate both myeloid and erythroid cells. We propose that Pax3 lineage haematopoietic cells should be investigated to better understand normal haematopoietic development from different mesodermal derivatives. Further, genetic alterations of Pax3 lineage haematopoietic cells should be investigated for their potential to cause haematopoietic malignancies. [ABSTRACT FROM AUTHOR]

Despite great progress in treating atrial fibrillation (AF), especially with the development of increasingly effective invasive techniques for AF ablation, many unanswered questions remain regarding the pathogenic mechanism of the arrhythmia and its prevention methods. The development of AF is based on anatomical and functional alterations in the cardiomyocyte resulting from altered ionic fluxes and cardiomyocyte electrophysiology. Electric instability and electrical remodeling underlying the arrhythmia may result from oxidative stress, also caused by possible mitochondrial dysfunction. The role of mitochondrial dysfunction in the pathogenesis of AF is not yet fully elucidated; however, the reduction in AF burden after therapeutic interventions that improve mitochondrial fitness tends to support this concept. This selected review aims to summarize the mechanisms of mitochondrial dysfunction related to AF and the current pharmacological treatment options that target mitochondria to prevent or improve the outcome of AF. [ABSTRACT FROM AUTHOR]

We previously described three patients affected by metastatic colorectal cancer (mCRC) who experienced spontaneous tumour shrinkage during severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Thereafter, the patients were closely monitored and no systemic treatments were applied. Here, we report follow-up clinical information about these patients as well as genetic characterization of their primary tumours through the TruSigt™Oncology 500 Next Generation Sequencing test targeting 523 cancer-relevant genes. An Illumina NovaSeq 6000 platform was used to perform sequencing. Time-to-progression was 23 and 2 months, respectively, in Patients 2 and 3 while it was not reached in Patient 1. Patients 1 and 2 had the greatest anti-SARS-CoV-2 IgG titres. Assessment of genetic landscapes evidenced common mutation in BARD1 gene (p.Val507Met) in Patients 1 and 2. Although our report is descriptive in its nature, we suggest that complex and unexplored interactions between genetic background and components of the immune response to SARS-CoV-2 infection could be responsible of unexpected rare mCRC shrinkage.

Placement of traditional transvenous implantable cardioverter defibrillator (ICD) system in low-weight children is often difficult because of their vessel size, the elevated risk of lead malfunction and failure, children’s growth and various anatomic constraints, creating the need for alternative solutions. Subcutaneous array leads combined with an abdominally placed ICD device can minimize the surgical approach. In this case series, we analyse the data behind indications for subcutaneous finger cardioverter defibrillator (SFCD) and discuss the preliminary clinical experience in low-weight children. We considered 4 consecutive children (mean age 3.9 years, range 3-5.5 years, mean body weight 17.6 Kg, range 14-23 Kg) who underwent SFCD implant from April 2016 to August 2020. All patients showed a good compliance to the device system with no complications (infections or skin erosions). No patients experienced in the observation period (mean time 44.5±21.5 months) sustained ventricular arrhythmias requiring shocks. No inappropriate shocks released by the device occurred. No significant changes were observed in LET (lowest energy tested) performed around 24 months of follow-up. All patients showed a good compliance and stable atrio-ventricular sensing and pacing thresholds. In smaller children in whom a transvenous approach is not feasible or not possible for anatomic reasons, the SFCD appears to be a safe method to prevent SCD with little surgical trauma and preservation of an intact vascular system, providing an adequate bridge to transvenous ICD or subcutaneous ICD implant late in the life.

Obstruction of the superior vena cava represents an under-recognized cause of chylothorax in the adult population. Our case report describes the successful conservative management of chylothorax due to bilateral superior vena cava obstruction in an adult patient with complex congenital heart disease. (Level of Difficulty: Beginner.)

Introduction: Early‐onset atrial fibrillation (AF) has already been observed in approximately 2% of patients with genetically proven long QT syndrome (LQTS). This frequency is higher than population‐based estimates of early‐onset AF. However, the concomitant expression of AF in LQTS is likely underestimated. The purpose of this study was to examine the clinical presentation, genetic background, and outcomes of a cohort of patients with LQTS and early‐onset AF referred to a single tertiary center. Methods: Twenty‐seven patients diagnosed with congenital LQTS were included in the study based on the documentation of early‐onset (age ≤50 years) clinical or subclinical AF episodes in all available medical records, including standard electrocardiograms, wearable monitor or cardiac implantable electronic devices. Results: Seventeen patients experienced clinical AF during the follow‐up period. Subclinical AF was detected in 10 patients through insertable or wearable cardiac monitors. In our series, the mean heart rate during AF episodes was found to be relatively low despite the patients' young age and the low or minimal effective doses of beta‐blockers used for QTc interval control. All patients exhibiting LQTS and early‐onset AF were genotype positive, carrying mutations in the KCNQ1 (66%), KCNH2, KCNE1, and SCN5A genes. Notably, most of these patients carried the same p.(R231C) mutation in the KCNQ1 gene (59%) and were from the same families, suggesting concurrent expression of familial AF and LQTS. Conclusion: LQTS patients are prone to developing clinical and subclinical AF, even at a younger age. The occurrence of early‐onset AF in the LQTS population could be more frequent than previously assumed. AF should be considered as a potential dysrhythmia related to LQTS. Our study emphasizes the importance of carefully researching clinical and/or subclinical episodes of AF through strict heart rhythm monitoring in the LQTS population. [ABSTRACT FROM AUTHOR]

Background: Cardiac arrest (CA) is a life-critical condition. Patients who survive after CA go into a defined post-cardiac arrest syndrome (PCAS). In this clinical context, the role of the echocardiogram in recent years has become increasingly important to assess the causes of arrest, the prognosis, and any direct and indirect complications dependent on cardiopulmonary resuscitation (CPR) maneu-vers. Methods: We have conduct a narrative revision of literature. Results: The aim of our review is to evaluate the increasingly important role of the transthoracic and transesophageal echocardiogram in the CA phase and especially post-arrest, analyzing the data already present in the literature. Conclusion: Transthoracic and transesophageal echocardiogram in the CA phase take on important diagnostic and prognostic role. [ABSTRACT FROM AUTHOR]

Abstract This paper presents a dual-band resonant cavity in Substrate Integrated Waveguide (SIW) technology to operate in the range from 3.3 GHz to 4.2 GHz, spectrum considered for the Fifth Generation (5G) network. The cavity was designed to operate at 3.4 GHz and 4.0 GHz. To achieve different states, a jumper and a PIN diode switch are considered as switching elements, connecting and disconnecting the bottom and the upper walls of the SIW resonant cavity through a metal post inserted inside its internal volume. A fitting equation is proposed to predict the maximum resonance frequency caused by the insertion of a single metal post inside the internal volume of the SIW resonant cavity and a new low-cost biasing network is designed on a thin dielectric substrate allocated on the upper wall of the SIW resonant cavity, using transmissions lines and a single capacitor, reducing the final cost and the insertion losses. Good agreement was observed among the fitting equation, computational electromagnetic simulations, and experimental results, validating the proposed methods.

Glyphosate is a widely used herbicide, and its protonation and deprotonation sites are fundamental to understanding its properties. In this work, the sodiated, protonated, and deprotonated glyphosate were evaluated in the gas phase by infrared multiple photon dissociation spectroscopy to determine the exact nature of these coordination, protonation, and deprotonation states in the gas phase. In this context, Natural Bond Orbital analyses were carried out to unravel interactions that govern glyphosate (de)protonation states in the gas phase. The solvent effect on the protonation/deprotonation equilibria was also investigated by implicit (Solvation Model Based on Density and polarizable continuum models) and explicit solvation models (Monte Carlo and Molecular Dynamics simulations). These results show that glyphosate is protonated in the phosphonate group in the gas phase because of the strong hydrogen bond between the carboxylic oxygen (O7) and the protonated phosphonate group (O8–H19), while the most stable species in water is protonated at the amino group because of the preferential interaction of the NH2+ group and the solvent water molecules. Similarly, deprotonated glyphosate [Glyp−H]− was shown to be deprotonated at the phosphonate group in the gas phase but not in solution, also because of the preferential solvation of the NH2+ group present in the other deprotomers. Therefore, these results show that the stabilization of the protonated amino group by the solvent molecules is the governing factor of the (de)protonation equilibrium of glyphosate in water. [ABSTRACT FROM AUTHOR]