Prognostic role of exercise intensity in familial Filamin C truncating variants.

Background: Truncating variants in the Filamin C () gene are causative of highly arrhythmogenic cardiomyopathies. Guidelines remain controversial concerning competitive and high-intensity sports for carriers. Indeed, the impact of high-intensity exercise on individuals carrying these variants remains poorly understood.

Clustering in dilated cardiomyopathy at initial evaluation: An effective tool for clinical stratification.

Aims: Dilated cardiomyopathy (DCM) has a highly variable presentation and disease course. Current stratification strategies are complex and require multimodality evaluation. Using machine learning (ML) on a large dataset obtained at first cardiological evaluation, this study aims to identify specific DCM subgroups.

Genetic and Phenotypic Characterization of Nexilin (NEXN)-Related Cardiomyopathy: Results From a Multicentric Study.

Background: Nexilin (NEXN)-related cardiomyopathies (CMPs) are largely unexplored.

Objectives: This study investigated the causative role of NEXN in CMPs, examining its phenotypic expression and prognostic profile.

Methods: Twelve referral centers collected phenotypic/genotypic data of patients with NEXN variants.

Major arrhythmias in non-dilated left ventricular cardiomyopathy: a novel prediction score.

Background And Aims: The prediction of the first major arrhythmic event (MAE) is still an unmet need in the recently defined scenario of non-dilated left ventricular cardiomyopathy (NDLVC).

Methods: A cohort of 337 patients with NDLVC and no history of MAE was retrospectively identified at two large centres. Patient-tailored diagnostic workup included cardiac magnetic resonance (CMR), endomyocardial biopsy, and genetic testing.

Implications of obesity on clinical outcomes in acute decompensated heart failure across the left ventricular ejection fraction spectrum and right ventricular dysfunction.

Aims: The implications of obesity on phenotype presentation and outcomes in acute decompensated heart failure (ADHF) are relatively unexplored. The aim of this study was to investigate the characteristics and prognostic implications related to obesity in ADHF, according to left ventricular and right ventricular function.

Methods: Consecutive patients hospitalized for ADHF were retrospectively enrolled.

The Diagnostic and Prognostic Value of the 12-Lead ECG in Arrhythmogenic Left Ventricular Cardiomyopathy.

Background: Electrocardiographic findings in arrhythmogenic left ventricular cardiomyopathy (ALVC) have been limited to small studies.

Objectives: The authors aimed to analyze the electrocardiogram (ECG) characteristics of ALVC, to correlate ECG with cardiac magnetic resonance and genetic data, and to evaluate its prognostic value.

Methods: We reviewed data of 125 consecutive patients with ALVC (81.

Fenotypic expressions and clinical manifestations of arrhythmogenic cardiomyopathy.

Arrhythmogenic cardiomyopathy (ACM) is a cardiac disorder characterized by structural alterations of the myocardium, which predisposes individuals to ventricular arrhythmias and increases the risk of sudden cardiac death. Initially described as arrhythmogenic right ventricular cardiomyopathy, the involvement of the left ventricle (LV) has been subsequently recognized, leading to the classification of various phenotypes under LV non-dilated cardiomyopathy. The clinical spectrum of ACM ranges from life-threatening ventricular arrhythmias to overt heart failure, sometimes presenting with acute myocarditis-like episodes and extracardiac symptoms, further contributing to the disease's heterogeneity.

Exercise Intensity and Cardiac Disease Development in carriers of Titin variants.

Aims: While the exacerbating effect of physical exercise and its correlation with arrhythmic outcomes have been demonstrated for Arrhythmogenic Right Ventricular Cardiomyopathy, the impact of physical exercise on other forms of cardiomyopathies is poorly characterized. This study aimed to investigate the relationship between lifelong exercise intensity and the development of cardiac manifestations in subjects carrying a Likely Pathogenic (LP) or Pathogenic (P) truncating variant of titin (TTNtv).

Methods: TTNtv carriers - patients and family members - were interviewed regarding their exercise habits from birth until diagnosis (type of activity, hours/week, weeks/months, months/years, and number of years of exercise).

Critical analysis of the 2023 ESC guidelines on cardiomyopathy management.

The first European Society of Cardiology (ESC) guidelines on the management of cardiomyopathies (CMPs), published 1 year ago, remain highly relevant. These guidelines provide a comprehensive framework to manage the complexity of CMPs, consolidating previous approaches. All CMPs are now addressed systematically in one document.

Critical approach to the 2023 European Society of Cardiology guidelines on cardiomyopathies: a document by the Working Group on Myocardial and Pericardial Diseases of the Italian Society of Cardiology.

Last year, the European Society of Cardiology (ESC) published the first guidelines to comprehensively address the management of cardiomyopathies. This document by the Working Group on Myocardial and Pericardial Diseases of the Italian Society of Cardiology aims at highlighting the most relevant messages and novelties introduced by these guidelines for the management of patients affected by cardiomyopathies. Five main messages are summarized: the key role of the phenotype, the new classification of cardiomyopathies provided in the ESC guidelines, the importance given to new techniques such as cardiac magnetic resonance (CMR) and genetic testing, the newly provided recommendations given on sport activities and finally how the importance of follow-up evaluations is highlighted.

Arrhythmic Risk Stratification of Carriers of Filamin C Truncating Variants.

Importance: Filamin C truncating variants (FLNCtv) are a rare cause of cardiomyopathy with heterogeneous phenotypic presentations. Despite a high incidence of life-threatening ventricular arrhythmias and sudden cardiac death (SCD), reliable risk predictors to stratify carriers of FLNCtv are lacking.

Objective: To determine factors predictive of SCD/major ventricular arrhythmias (MVA) in carriers of FLNCtv.

Predicting survival in patients with severe heart failure: Risk score validation in the HELP-HF cohort.

Aims: Accurate selection of patients with severe heart failure (HF) who might benefit from advanced therapies is crucial. The present study investigates the performance of the available risk scores aimed at predicting the risk of mortality in patients with severe HF.

Methods And Results: The risk of 1-year mortality was estimated in patients with severe HF enrolled in the HELP-HF cohort according to the MAGGIC, 3-CHF, ADHF/NT-proBNP, and GWTG-HF risk scores, the number of criteria of the 2018 HFA-ESC definition of advanced HF, I NEED HELP markers, domains fulfilled of the 2019 HFA-ESC definition of frailty, the frailty index, and the INTERMACS profile.

Hot Phases Cardiomyopathy: Pathophysiology, Diagnostic Challenges, and Emerging Therapies.

Purpose Of Review: Hot phases are a challenging clinical presentation in arrhythmogenic cardiomyopathy (ACM), marked by acute chest pain and elevated cardiac troponins in the absence of obstructive coronary disease. These episodes manifest as myocarditis and primarily affect young patients, contributing to a heightened risk of life-threatening arrhythmias and potential disease progression. This review aims to synthesize recent research on the pathophysiology, diagnostic challenges, and therapeutic management of hot phases in ACM.

In-hospital evolution of secondary mitral regurgitation in acutely decompensated heart failure.

Aims: Secondary mitral regurgitation (MR) negatively affects prognosis in acutely decompensated heart failure (ADHF), but can be rapidly sensitive to changes in volume status and medical interventions. We sought to assess the evolution of secondary MR in patients hospitalized for ADHF and its prognostic implications.

Methods: We retrospectively enrolled 782 patients admitted for ADHF with at least two in-hospital echocardiographic evaluations of MR.

Abnormal conduction-induced cardiomyopathy: a poorly explored entity.

A dyssynchronous biventricular activation, which can be determined by left bundle branch block, chronic right ventricular pacing, frequent premature ventricular complexes, or pre-excitation, can cause a global abnormal contractility, thus leading to systolic dysfunction and left ventricular remodelling in a unique nosological entities: abnormal conduction-induced cardiomyopathies. In this clinical scenario, the mainstay therapy is eliminating or improving LV dyssynchrony, removing the trigger. This usually ensures the improvement and even recovery of cardiac geometry and left ventricular function, especially in the absence of genetic background.

Prognostic role of mitral regurgitation in patients with advanced heart failure.

Aim: The impact of mitral regurgitation (MR) in patients with advanced heart failure (HF) is poorly known. We aimed to evaluate the impact of MR on clinical outcomes of a real-world, contemporary, multicentre population with advanced HF.

Methods: The HELP-HF registry enrolled patients with HF and at least one "I NEED HELP" criterion, at four Italian centres between January 2020 and November 2021.

Clinical burden and predictors of non-cardiovascular mortality and morbidity in advanced heart failure.

Background: The changing demographic of heart failure (HF) increases the exposure to non-cardiovascular (non-CV) events. We investigated the distribution of non-CV mortality/morbidity and the characteristics associated with higher risk of non-CV events in patients with advanced HF.

Methods: Patients from the HELP-HF registry were stratified according to the number of 2018 HFA-ESC criteria for advanced HF.

Guideline-directed medical therapy in severe heart failure with reduced ejection fraction: An analysis from the HELP-HF registry.

Aim: Persistent symptoms despite guideline-directed medical therapy (GDMT) and poor tolerance of GDMT are hallmarks of patients with advanced heart failure (HF) with reduced ejection fraction (HFrEF). However, real-world data on GDMT use, dose, and prognostic implications are lacking.

Methods And Results: We included 699 consecutive patients with HFrEF and at least one 'I NEED HELP' marker for advanced HF enrolled in a multicentre registry.