Influence of age and sex on the diagnostic yield of inherited cardiac conditions in sudden arrhythmic death syndrome decedents.

Aims: Sudden arrhythmic death syndrome (SADS) refers to a sudden death, which remains unexplained despite comprehensive post-mortem examination and a toxicological screen. We aimed to investigate the impact of age and sex on the overall diagnostic yield and underlying aetiology in decedents with SADS using a combined approach of familial evaluation (FE) and molecular autopsy (MA).

Methods And Results: Consecutive referrals to a single centre for FE only, MA only or both, following a SADS death were included.

Corrigendum to [Int J Cardiol Congenit Heart Dis 9 September 2022 100394].

[This corrects the article DOI: 10.1016/j.ijcchd.

Diagnosis and management of peripartum cardiomyopathy and recurrence risk.

Peripartum cardiomyopathy (PPCM) is a rare, but serious condition, with a non-negligible risk of adverse events. Several risk factors for PPCM have been individuated over the years, including Afro-American ethnicity, preeclampsia, advanced maternal age, genetic predisposition, multiparity, twin pregnancy, obesity, smoking and diabetes. However, PPCM pathophysiology is still poorly understood, thus making it challenging to develop disease specific therapies.

Pulmonary arterial hypertension with left to right shunts: When to treat and/or close?

Pulmonary arterial hypertension (PAH) is defined as increase in mean pulmonary arterial pressure and pulmonary vascular resistance (PVR). It can be associated with congenital heart disease (CHD) with the following subtypes: 1) uncorrected left-to-right (L-R) intracardiac shunt leading to overload of the pulmonary circulation and a progressive increase of PVR; 2) Eisenmenger syndrome, appearing when a large post-tricuspid shunt is left uncorrected and pulmonary vascular disease (PVD) is severe, so the shunt becomes bidirectional or right-to-left, causing cyanosis; 3) PAH after shunt closure, when PVR arises after a defect correction; and 4) PAH associated with small or coincidental defects. While the treatment of patients with Eisenmenger syndrome is well established, the treatment of patients with PAH in whom there is a L-R shunt (with no cyanosis) remains unclear and requires expertise.

Corrigendum to "Exercise ventilatory reserve predicts survival in adult congenital heart disease associated pulmonary arterial hypertension with Eisenmenger physiology" [Int J Cardiol Congenit Heart Dis, Volume 7, March 2022, 100331].

[This corrects the article DOI: 10.1016/j.ijcchd.

Cardiac catheterization in pulmonary arterial hypertension: Tips and tricks to enhance diagnosis and guide therapy.

Cardiac catheterization (CC) is essential for the diagnosis of pulmonary hypertension (PH), and for its characterisation. It allows distinction between pre- and post-capillary PH which, when integrated with other non-invasive data, facilitates classification into one of the 5 diagnostic groups defined by international PH guidelines. CC also provides valuable information for the risk stratification of patients with PH, guiding management and the type and intensity of treatment.

Clinical importance of patient-reported outcome measures in severe asthma: results from U-BIOPRED.

Rationale: Knowledge about the clinical importance of patient-reported outcome measures (PROMs) in severe asthma is limited.

Objectives: To assess whether and to what extent asthma exacerbations affect changes in PROMS over time and asthma-specific PROMs can predict exacerbations in adult patients with severe asthma in usual care.

Methods: Data of 421 patients with severe asthma (62% female; mean age 51.

TNIK: A redox sensor in endothelial cell permeability.

Dysregulation of endothelial barrier integrity can lead to vascular leak and potentially fatal oedema. TNF-α controls endothelial permeability during inflammation and requires the actin organizing Ezrin-Radixin-Moesin (ERM) proteins. We identified TRAF2 and NCK-interacting kinase (TNIK) as a kinase directly phosphorylating and activating ERM, specifically at the plasma membrane of primary human endothelial cells.

Rising mortality rates linked to type-2 diabetes and obesity in the United States: An observational analysis from 1999 to 2022.

Background: The prevalence of type 2 diabetes (T2D) and obesity are increasing in the United States. However, population-level data for mortality trends due to T2D and obesity are limited. This study aims to assess these death trends among adults in the United States categorized by sex, race, and geographical location.

Cervical Artery Tortuosity Is Associated With Dissection Occurrence and Late Recurrence: A Nested Case-Control Study.

Background: The pathogenesis of spontaneous cervical artery dissection remains unclear, and no established predictors of recurrence exist. Our goal was to investigate the potential association between cervical artery tortuosity, a characteristic of patients with connective tissue disorder, and spontaneous cervical artery dissection.

Methods: The ReSect study (Risk Factors for Recurrent Cervical Artery Dissection) is an observational study that invited all spontaneous cervical artery dissection patients treated at the Innsbruck University Hospital between 1996 and 2018 for clinical and radiological follow-up.

Social inequalities in childhood asthma.

Asthma is a complex, heterogeneous condition, broadly characterized by chronic airway inflammation with variable expiratory airflow limitation, but with several subtypes underpinned by different (although likely overlapping) pathological mechanisms. It is one of the most common chronic diseases of childhood and represents a significant cost for healthcare systems and affected families. Evidence suggests that a disproportionate proportion of this burden falls on families from disadvantaged socioeconomic circumstances (SECs).

Extreme temperature increases the risk of COPD morbimortality: A systematic review and meta-analysis.

Introduction: This systematic review examines how extreme temperatures impact chronic obstructive pulmonary disease (COPD) morbidity and mortality, focusing on identifying vulnerable subpopulations.

Methods: We conducted a systematic literature search from January 1, 2000, to November 6, 2024, across databases like PubMed, MEDLINE and EMBASE, Web of Science, and Scopus, focusing on observational studies that quantitatively defined extreme temperatures and their impacts on COPD morbidity and mortality. Out of 3140 records, 25 studies met the inclusion criteria.

"We Could Hold Our Own Here at Home": Longitudinal Experience of COVID-19 Lockdowns in Parents With Children Affected With Interstitial Lung Disease.

The global health emergency of COVID-19 in early 2020 placed much of the population under quarantine. Interstitial Lung Disease in childhood (chILD) was recommended to be a pediatric clinically extremely vulnerable (CEV) group in April 2020 for shielding due to the unknown health consequences of COVID-19 in children with chronic respiratory conditions. This qualitative longitudinal research study explores how chILD parents in the UK experienced COVID-19 lockdown from over two interview time points.

Protective effect of UDCA against IL-11- induced cardiac fibrosis is mediated by TGR5 signalling.

Introduction: Cardiac fibrosis occurs in a wide range of cardiac diseases and is characterised by the transdifferentiation of cardiac fibroblasts into myofibroblasts these cells produce large quantities of extracellular matrix, resulting in myocardial scar. The profibrotic process is multi-factorial, meaning identification of effective treatments has been limited. The antifibrotic effect of the bile acid ursodeoxycholic acid (UDCA) is established in cases of liver fibrosis however its mechanism and role in cardiac fibrosis is less well understood.

Development of a natural rubber latex-based biodevice with mesenchymal stem cells as a potential treatment for skeletal muscle regeneration in gestational diabetes-induced myopathy.

Women with gestational diabetes mellitus show a high risk of developing Gestational Diabetes Induced Myopathy (GDiM). GDiM is characterized by significant pelvic floor skeletal muscle atrophy and urinary incontinence. This study aimed to develop a natural rubber latex (NRL) based biodevice with mesenchymal/stromal stem cells (MSCs) for skeletal muscle regeneration for women with GDiM.

Enhancing stroke risk stratification in atrial fibrillation through non-Newtonian blood modelling and Gaussian process emulation.

Atrial fibrillation (AF) is the most common heart arrhythmia, linked to a five-fold increase in stroke risk. The left atrial appendage (LAA), prone to blood stasis, is a common thrombus formation site in AF patients. The LAA can be classified into four morphologies: broccoli, cactus, chicken wing and windsock.

From bits to bedside: entering the age of digital twins in cardiac electrophysiology.

This State of the Future Review describes and discusses the potential transformative power of digital twins in cardiac electrophysiology. In this 'big picture' approach, we explore the evolution of mechanistic modelling based digital twins, their current and immediate clinical applications, and envision a future where continuous updates, advanced calibration, and seamless data integration redefine clinical practice of cardiac electrophysiology. Our aim is to inspire researchers and clinicians to embrace the extraordinary possibilities that digital twins offer in the pursuit of precision medicine.

Effects of nintedanib on circulating biomarkers of idiopathic pulmonary fibrosis.

Background: Biomarkers that change in response to nintedanib in subjects with idiopathic pulmonary fibrosis (IPF) would be valuable. We investigated the effects of nintedanib on circulating biomarkers in subjects with IPF in the INMARK trial.

Methods: Subjects with IPF were randomised 1:2 to receive nintedanib 150 mg twice daily or placebo for 12 weeks, after which all patients received open-label nintedanib for 40 weeks.

Proteomic profiling of bronchoalveolar lavage fluid uncovers protein clusters linked to survival in idiopathic forms of interstitial lung disease.

Background: Idiopathic interstitial pneumonias (IIPs), such as idiopathic pulmonary fibrosis and interstitial pneumonia with autoimmune features, present diagnostic and therapeutic challenges due to their heterogeneous nature. This study aimed to identify intrinsic molecular signatures within the lung microenvironment of these IIPs through proteomic analysis of bronchoalveolar lavage fluid (BALF).

Methods: Patients with IIP (n=23) underwent comprehensive clinical evaluation including pre-treatment bronchoscopy and were compared with controls without lung disease (n=5).

Nationwide Cross-Sectional Analysis of Mortality Trends in Patients with Sarcoidosis and Non-Ischemic Cardiovascular Disease-The Impact of Gender, Ethnicity, Geographical Location, and COVID-19 Pandemic.

The epidemiological data regarding mortality rates of adults with sarcoidosis and non-ischemic cardiovascular disease (CVD) are limited. A retrospective observational analysis was conducted to identify trends and disparities related to sarcoidosis and non-ischemic cardiovascular disease mortality among the adult US population from 1999 to 2022. We used the Centers for Disease Control and Prevention (CDC) WONDER database to extract death certificate data for the adult US population (≥25 years).

Management of Acute Ischemic Stroke Following Transcatheter Aortic Valve Implantation: A Systematic Review and Multidisciplinary Treatment Recommendations.

: Acute ischemic stroke is an uncommon but potentially devastating complication of Transcatheter Aortic Valve Implantation (TAVI). Despite improvements in device technology and procedural techniques, stroke rates have remained stable, with cerebral embolic protection devices demonstrating only limited efficacy to date. Therefore, the management of acute ischemic stroke complicating TAVI (AISCT) remains a key priority.

Identifying potential predictors of surgical site infection risk following cardiac surgery: a scoping review.

Objectives: This scoping review was undertaken to identify risk predictions models and preoperative predictors of surgical site infection (SSI) in adult cardiac surgery. A particular focus was on the identification of novel predictors that could underpin the future development of a risk prediction model to identify individuals at high-risk of SSI, and therefore guide a national SSI prevention strategy.

Methods: A scoping review to systematically identify and map out existing research evidence on preoperative predictors of SSI was conducted in two stages.

Prevalence and Clinical Correlates of Radiologically Detected Coronary Artery Disease in COPD: A Cross-Sectional Observational Study.

Rationale: Unrecognised coronary artery disease (CAD) may contribute to adverse outcomes in chronic obstructive pulmonary disease (COPD). Improved identification of at-risk groups could inform better preventative care. We aimed to evaluate the burden and relationships of radiologically detectable CAD in COPD, establish frequency of occult disease, and examine potential cardiovascular screening methods.