What Else Is Needed to Improve Survival from Out-of-Hospital Cardiac Arrest to Hospital Admission? Data from a Prospective Registry for the Years 2020-2023 in the Italian Province of Varese.

Around the world, data on out-of-hospital cardiac arrest (OHCA) are heterogeneous in terms of outcomes and reporting, and not all registries follow the Utstein recommendations for uniform OHCA data collection. This study reports data on OHCA occurring in recent years in a limited territory to analyze, in a homogenous setting, the circumstances and interventions affecting survival to hospital admission. OHCA data from the province of Varese for the years 2020-2022 were extracted from a prospective registry. For survival to hospital admission, the impact of pandemic waves and variables known to affect survival was evaluated both in the overall population and in the subgroup of patients in whom cardiopulmonary resuscitation (CPR) was initiated or continued by the emergency medical service (EMS). Overall, 3263 OHCAs occurred mainly at home (88%), with a time to intervention of 13.7 min, which was significantly longer during lockdown (15.7 min). Bystanders performed CPR in 22% of the cases and used automatic external defibrillator (AED) in 2.2% of the cases. Overall survival to hospital admission was 7.7%. In the multivariate analysis, in the general population, occurrence near a public building (OR 1.92), the presence of witnesses (OR 2.65), and a shockable rhythm (OR 7.04) were independent predictors of survival to hospital admission, whereas age (OR 0.97) and occurrence during a pandemic wave (OR 0.62) were associated with significantly worse survival to hospital admission. In the group of patients who received CPR, AED shock by bystanders was the only independent predictor of survival (OR 3.14) to hospital admission. Among other factors, early defibrillation was of crucial importance to improve survival to hospital admission in possibly rescuable patients. The occurrence of OHCA during pandemic waves was associated with longer intervention time and worse survival to hospital admission.

Desmoplakin cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy: two distinct forms of cardiomyopathy?

The confirmation of a hypothesis that desmoplakin-related (DSP) cardiomyopathy could represent a distinct clinical entity from the classical, RV-dominant, form of arrhythmogenic cardiomyopathy (ACM), most frequently caused by PKP2 mutations, would without any shadow of doubt signify a turning point in the history of this disease. The concept of gene-specific diseases underneath the umbrella diagnosis of ACM would bring fundamental changes not only in the clinical, diagnostic and therapeutic approach, but also in terms of risk stratification, pushing the scientific community towards a more patient-centered view of the disease, similarly to what has already been done in other inherited arrhythmogenic disease (e.g., long QT syndrome [LQTS]). We provide a state-of-the-art review, starting with a brief historical framework to give the necessary context and better focus the question. Then, we proceed with a novel, genotype-to-phenotype-based comparison of the most important aspects of DSP-related cardiomyopathy with the classical, RV-dominant ACM: this allows us to ascertain not only that the differences between the forms exist, but are also clinically relevant and actionable, leading to the underrecognition of the atypical, DSP-related, LV-dominant forms when applying the current diagnostic criteria. These findings will usher an exciting era, in which the scientific community will try to answer a range of questions, starting from the reasons why different desmosomal mutations cause such different phenotypes.

Arrhythmic Mitral Valve Prolapse: Introducing an Era of Multimodality Imaging-Based Diagnosis and Risk Stratification.

Mitral valve prolapse is a common cardiac condition, with an estimated prevalence between 1% and 3%. Most patients have a benign course, but ever since its initial description mitral valve prolapse has been associated to sudden cardiac death. Although the causal relationship between mitral valve prolapse and sudden cardiac death has never been clearly demonstrated, different factors have been implicated in arrhythmogenesis in patients with mitral valve prolapse. In this work, we offer a comprehensive overview of the etiology and the genetic background, epidemiology, pathophysiology, and we focus on the state-of-the-art imaging-based diagnosis of mitral valve prolapse. Going beyond the classical, well-described clinical factors, such as young age, female gender and auscultatory findings, we investigate multimodality imaging features, such as alterations of anatomy and function of the mitral valve and its leaflets, the structural and contractile anomalies of the myocardium, all of which have been associated to sudden cardiac death.