Prognostic Role of Myocarditis-Like Episodes and Their Treatment in Patients With Pathogenic Desmoplakin Variants.

Background: Inflammatory, myocarditis-like episodes precede and are associated with higher risk of sustained ventricular arrhythmias and heart failure in patients with pathogenic or likely pathogenic desmoplakin (DSP) variants. Whether the recurrence and treatment of myocarditis-like episodes influence the outcomes in this population is unknown. This study aimed to assess the prognostic impact of the recurrence and treatment of myocarditis-like episodes in patients with pathogenic or likely pathogenic DSP variants.

Accelerated vascular ageing after COVID-19 infection: the CARTESIAN study.

Background And Aims: Increasing evidence suggests that COVID-19 survivors experience long-term cardiovascular complications possibly through development of vascular damage. The study aimed to investigate whether accelerated vascular ageing occurs after COVID-19 infection, and if so, identify its determinants.

Methods: This prospective, multicentric, cohort study, included 34 centres in 16 countries worldwide, in 4 groups of participants-COVID-19-negative controls (ⅰ) and three groups of individuals with recent (6 ± 3 months) exposure to SARS-CoV-2: not hospitalized (ⅱ), hospitalized in general wards (ⅲ), and hospitalized in intensive care units (ⅳ).

Diagnostic Criteria and Disease Staging for Desmoplakin Cardiomyopathy.

Background: Desmoplakin (DSP) cardiomyopathy, caused by variants in the gene , is a unique subtype of cardiomyopathy distinct from typical dilated or arrhythmogenic right ventricular cardiomyopathies. Specific diagnostic and disease staging criteria have yet to be developed for DSP cardiomyopathy.

Objective: Utilizing a large cohort of DSP cardiomyopathy patients and their genotype-positive family members, this study aims to develop diagnostic and disease staging criteria for DSP cardiomyopathy.

Strategies to enhance recruitment of female participants to cardiovascular research: a joint British Cardiovascular Societies' consensus document in collaboration with the British Heart Foundation Clinical Research Collaborative.

Despite significant progress in cardiovascular pharmacotherapy and interventional strategies, cardiovascular disease (CVD) remains the leading cause of morbidity and mortality among females in the UK and worldwide. This might be due to lack of robust evidence in the best care of females with CVD related to under-representation of females in clinical trials (females accounting for <30% of trial participants). Recently, the British Cardiovascular Society (BCS), together with the affiliated societies, put together a consensus document specifically describing the current status on the sex differences in each of the major disease areas and proposed strategies/actionable points to overcome the barriers in access to diagnosis and treatment of CVD among females.

Proteomic Analysis of Valsartan for Attenuating Disease Evolution in Early Sarcomeric Hypertrophic Cardiomyopathy (VANISH) Clinical Trial.

Background: In hypertrophic cardiomyopathy (HCM), the mechanisms through which pathogenic sarcomere variants (G+) lead to left ventricular hypertrophy (LVH) are not understood.

Methods: VANISH (Valsartan for Attenuating Disease Evolution in Early Sarcomeric Hypertrophic Cardiomyopathy) was a multicenter, double-blind, placebo-controlled, randomized trial testing valsartan's ability to attenuate phenotypic progression in early sarcomeric (G+LVH+) and subclinical HCM (G+LVH‒). The outcome was a composite z-score reflecting change in cardiac remodeling from baseline to year 2 (end of study).

Peak frequency can be effectively used to characterize scar in atrial fibrillation.

Background: Characterizing atrial fibrillation (AF) substrate can guide ablation strategies.

Objective: A novel parameter, peak frequency (PF), was evaluated in its ability to characterize the substrate in AF.

Methods: Patients undergoing persistent AF ablation were included.

NAXCARE: a clinical outcome registry for Naxos disease and related cardiocutaneous syndromes.

The NAXCARE (NAXos disease and Cardiocutaneous Assessment and Registry for Evaluation) is a global initiative designed to collect, store, and analyze clinical outcomes data on patients with Naxos disease and related cardiocutaneous syndromes (CCS). This registry aims to fill the gaps in clinical knowledge, enhance treatment approaches, and improve patient outcomes by systematically documenting disease progression, genetic profiles, and patient care pathways. The following methodology outlines the registry's design, data collection protocols, management, security measures, and anticipated contributions to research and clinical practice.

Outcomes of immediate sequential bilateral cataract surgeries (ISBCS) performed by trainees vs consultants: a two-armed cohort study.

Purpose: To compare outcomes of Immediate Sequential Bilateral Cataract Surgeries (ISBCS) performed by resident doctors versus consultant ophthalmologists at Moorfields Eye Hospital and its satellite centres.

Methods: The study reviewed ISBCS performed by phacoemulsification and intraocular lens implant, comparing resident ophthalmologists (Gr1) and consultant ophthalmologists (Gr2). Outcomes examined included complications, refraction (spherical equivalent (SE) > 0.

Clinical utility and implementation of polygenic risk scores for predicting cardiovascular disease: A clinical consensus statement of the ESC Council on Cardiovascular Genomics, the ESC Cardiovascular Risk Collaboration, and the European Association of Preventive Cardiology.

Genome-wide association studies have revealed hundreds of genetic variants associated with cardiovascular diseases (CVD). Polygenic risk scores (PRS) can capture this information in a single metric and hold promise for use in CVD risk prediction. Importantly, PRS information can reflect the causally mediated risk to which the individual is exposed throughout life.

Naxos Disease and Related Cardio-Cutaneous Syndromes.

Naxos disease is a rare autosomal recessive condition combining arrhythmogenic right ventricular cardiomyopathy, woolly hair, and palmoplantar keratoderma. The first identified causative variant was in the gene encoding the desmosomal protein plakoglobin. Naxos disease exhibits fibro-fatty myocardial replacement with immunohistological abnormalities in cardiac protein and signaling pathways, highlighting the role of inflammation and potential anti-inflammatory treatments.

Symptom evolution in individuals with ongoing symptomatic COVID-19 and post-COVID-19 syndrome after SARS-CoV-2 vaccination versus influenza vaccination.

Background: COVID-19 symptoms may persist beyond acute SARS-CoV-2 infection, as ongoing symptomatic COVID-19 [OSC] (symptom duration 4-12 weeks) and post-COVID syndrome [PCS] (symptom duration ≥12 weeks). Vaccination against SARS-CoV-2 decreases OSC/PCS in individuals subsequently infected with SARS-CoV-2 post-vaccination. Whether vaccination against SARS-CoV-2, or any other vaccinations (such as against influenza) affects symptoms in individuals already experiencing OSC/PCS, more than natural symptom evolution, is unknown.

Higher Aircraft Noise Exposure Is Linked to Worse Heart Structure and Function by Cardiovascular MRI.

Background: Aircraft noise is a growing concern for communities living near airports.

Objectives: This study aimed to explore the impact of aircraft noise on heart structure and function.

Methods: Nighttime aircraft noise levels (L) and weighted 24-hour day-evening-night aircraft noise levels (L) were provided by the UK Civil Aviation Authority for 2011.

Hypertrophic cardiomyopathy management: a systematic review of the clinical practice guidelines and recommendations.

Aims: In light of recent advances in imaging techniques, molecular understanding and therapeutic options in hypertrophic cardiomyopathy (HCM), we performed a systematic review of current guidelines for the diagnosis and management of HCM in order to identify consensus and discrepant areas in the clinical practice guidelines.

Methods And Results: We systematically reviewed the English language guidelines and recommendations for the management of HCM in adults. MEDLINE and EMBASE databases were searched for guidelines published in the last 10 years.

Key priorities for the implementation of the 2023 ESC Guidelines for the Management of Cardiomyopathies in low resource settings.

ESC Guidelines provide best practice, evidence-based recommendations for diagnosing and treating patients with cardiovascular diseases. It is not always possible for best practices to be followed, however, particularly in low-resource settings. To address this issue, a set of guideline-related documents were created to identify key priorities for users in these settings.

Challenges in Cardiomyopathy Gene Therapy Clinical Trial Design.

Gene therapy has emerged as a possible treatment for progressive, debilitating Mendelian cardiomyopathies with limited therapeutic options. This paper arises from discussions at the 2023 Cardiovascular Clinical Trialists Forum and highlights several challenges relevant to gene therapy clinical trials, including low prevalence and high phenotypic heterogeneity of Mendelian cardiomyopathies, outcome selection complexities and resulting regulatory uncertainty, and immune responses to the adeno-associated viral vectors that are being used in ongoing studies. Avenues to address these challenges such as natural history studies, external controls, novel regulatory pathways, and immunosuppression are discussed.

Device-Measured 24-Hour Movement Behaviors and Blood Pressure: A 6-Part Compositional Individual Participant Data Analysis in the ProPASS Consortium.

Background: Blood pressure (BP)-lowering effects of structured exercise are well-established. Effects of 24-hour movement behaviors captured in free-living settings have received less attention. This cross-sectional study investigated associations between a 24-hour behavior composition comprising 6 parts (sleeping, sedentary behavior, standing, slow walking, fast walking, and combined exercise-like activity [eg, running and cycling]) and systolic BP (SBP) and diastolic BP (DBP).

On preserving anatomical detail in statistical shape analysis for clustering: focus on left atrial appendage morphology.

Introduction: Statistical shape analysis (SSA) with clustering is often used to objectively define and categorise anatomical shape variations. However, studies until now have often focused on simplified anatomical reconstructions, despite the complexity of studied anatomies. This work aims to provide insights on the anatomical detail preservation required for SSA of highly diverse and complex anatomies, with particular focus on the left atrial appendage (LAA).

Associations between Aircraft Noise, Sleep, and Sleep-Wake Cycle: Actimetric Data from the UK Biobank Cohort near Four Major Airports.

Background: Nighttime aircraft noise may affect people's sleep, yet large-scale evidence using objective and subjective measures remains limited.

Objective: Our aim was to investigate associations between nighttime aircraft noise exposure and objectively measured sleep disturbance using a large UK cohort.

Methods: We used data from 105,770 UK Biobank cohort participants exposed and unexposed to aircraft noise who lived in 44 local authority districts near 4 international airports in England.

Dual immunoadsorption for ABO-incompatible heart transplantation in a 35 kg child.

Intraoperative anti-A/B immunoadsorption (ABO-IA) was recently introduced for ABO-incompatible (ABOi) heart transplantation. Here we report the first case of a patient transplanted with ABO-IA, that was of an age and weight that required two ABO-IA columns run in parallel, to enable the reduction in antibody titres to a sufficiently low level in the time available during implantation of the donor organ.

Adulthood adiposity affects cardiac structure and function in later life.

Background And Aims: Excess adiposity is associated with poorer cardiac function and adverse left ventricular (LV) remodelling. However, its importance over the adult life course on future cardiac structure and systolic and diastolic function is unknown.

Methods: A total of 1690 participants in the National Survey of Health and Development birth cohort underwent repeated adiposity [body mass index (BMI)/waist-to-hip ratio (WHR)] measurements over adulthood and investigation, including echocardiography at age 60-64 years.

Multidimensional Analysis of the Adult Human Heart in Health and Disease Using Hierarchical Phase-Contrast Tomography.

Background Current clinical imaging modalities such as CT and MRI provide resolution adequate to diagnose cardiovascular diseases but cannot depict detailed structural features in the heart across length scales. Hierarchical phase-contrast tomography (HiP-CT) uses fourth-generation synchrotron sources with improved x-ray brilliance and high energies to provide micron-resolution imaging of intact adult organs with unprecedented detail. Purpose To evaluate the capability of HiP-CT to depict the macro- to microanatomy of structurally normal and abnormal adult human hearts ex vivo.