Fulminant Myocarditis Presenting With Refractory Ventricular Fibrillation During BRAF/MEK Inhibitor Therapy.

Background: Myocarditis is a rare but potentially fatal complication of BRAF/MEK inhibitor therapy. Early detection through vigilant monitoring is crucial.

Case Summary: A 35-year-old woman with stage IIIB melanoma treated with adjuvant trametinib and dabrafenib suffered a cardiac arrest due to refractory ventricular fibrillation.

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.

Characteristics and outcomes of patients with heart failure and history of malignancy: Data from Swedish Heart Failure Registry and the National Cancer Register.

Aims: Whether a history of malignancy affects heart failure (HF) management and prognosis is unclear. In HF randomized controlled trials, enrolment has been limited to patients with a cancer diagnosis at least 2 years before screening. We investigated clinical profile, HF treatment patterns, and outcomes of patients with a history of cancer of >2 years.

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 crosstalk between immune activation and metabolism in heart failure. A scientific statement of the Heart Failure Association of the ESC.

A better understanding of additional mechanisms of heart failure (HF) progression may allow a different and more complete phenotyping of the disease and identification of novel therapeutic targets. Persistent latent myocardial inflammation/immune activation in HF may represent an attempt to restore tissue homeostasis in the failing heart, where cardiomyocytes and immune cells undergo metabolic reprogramming, which allows them to deal with decreased availability of nutrients and oxygen. This status can trigger a metabolic crosstalk between immune cells and cardiomyocytes which, depending on the outcome, can either perpetuate the maladaptive remodelling of the heart, or determine an adaptive response.

Machine-learning phenotyping of patients with functional mitral regurgitation undergoing transcatheter edge-to-edge repair: the MITRA-AI study.

Aims: Severe functional mitral regurgitation (FMR) may benefit from mitral transcatheter edge-to-edge repair (TEER), but selection of patients remains to be optimized.

Objectives: The aim of this study was to use machine-learning (ML) approaches to uncover concealed connections between clinical, echocardiographic, and haemodynamic data associated with patients' outcomes.

Methods And Results: Consecutive patients undergoing TEER from 2009 to 2020 were included in the MITRA-AI registry.

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.

Aetiological phenotypes of atrial and ventricular secondary tricuspid regurgitation and their prognostic implications: insights from the CARE-TR registry.

Aims: To report prevalence and clinical outcomes of different aetiological phenotypes of atrial and ventricular secondary tricuspid regurgitation (ASTR/VSTR).

Methods And Results: The Consecutive pAtients with seveRE Tricuspid Regurgitation evaluated in Heart Failure (HF) and Valve Clinics (CARE-TR) registry collected data from patients with at least severe tricuspid regurgitation (TR) enrolled at three Italian centres. The present analysis includes 648 patients with secondary TR, 22.

Integrated role of cardiac magnetic resonance and genetics in predicting left ventricular reverse remodelling in dilated and non-dilated cardiomyopathy.

Aims: Left ventricular reverse remodelling (LVRR) is a prognostic marker in patients with dilated (DCM) and non-dilated left ventricular cardiomyopathy (NDLVC). The utility of combining late gadolinium enhancement (LGE) and genetic testing in predicting LVRR in DCM/NDLVC remains a knowledge gap. This study aimed to assess an integrated approach including LGE data and genetics to predict LVRR in DCM/NDLVC patients.

Characteristics, Prognosis, and European Society of Cardiology and European Respiratory Society Risk Stratification in Patients With Obesity and Pulmonary Arterial Hypertension.

Background: The impact of obesity on pulmonary arterial hypertension (PAH) remains largely underexplored, with excess weight potentially masking symptoms and affecting the reliability of current risk stratification tools.

Research Question: What are the clinical characteristics and prognosis of patients with obesity and PAH, and how well do current risk stratification tools perform in this population?

Study Design And Methods: We retrospectively included patients with an incident PAH diagnosis enrolled at 10 European tertiary care centers for PAH management and compared patients with and without obesity, defined by a BMI of ≥ 30 kg/m. Univariable and multivariable Cox regression models were fitted to assess the association between obesity and 5-year all-cause mortality.

Prediction and prognostic role of left ventricular systolic dysfunction in family screening for dilated cardiomyopathy and non-dilated left ventricular cardiomyopathy.

Aims: The prognostic significance of detecting left ventricular (LV) systolic dysfunction during family screening programmes (FSPs) in relatives of probands affected by dilated (DCM) and non-dilated left ventricular (NDLVC) cardiomyopathies remain unclear. This study sought to evaluate the prognostic role of LV systolic dysfunction detection in relatives of DCM/NDLVC probands and to define the most accurate FSP.

Methods And Results: Baseline and follow-up data of first-degree relatives of probands affected by DCM/NDLVC were collected.

Sex differences in pulmonary arterial hypertension: Insights from the FOCUS-PAH registry.

Background: Despite the higher incidence of pulmonary arterial hypertension (PAH) in women, sex differences in terms of clinical presentation and prognosis are still uncertain. We assess the impact of sex in patients with PAH.

Methods: Sex differences were evaluated in the multicenter FOCUS-PAH registry, that included patients diagnosed with PAH at 7 tertiary care centers between 2001 and 2022.

How to handle polypharmacy in heart failure. A clinical consensus statement of the Heart Failure Association of the ESC.

The multiplicity of coexisting comorbidities affecting patients with heart failure (HF), together with the availability of multiple treatments improving prognosis in HF with reduced ejection fraction, has led to an increase in the number of prescribed medications to each patient. Polypharmacy is defined as the regular use of multiple medications, and over the last years has become an emerging aspect of HF care, particularly in older and frailer patients who are more frequently on multiple treatments, and are therefore more likely exposed to tolerability issues, drug-drug interactions and practical difficulties in management. Polypharmacy negatively affects adherence to treatment, and is associated with a higher risk of adverse drug reactions, impaired quality of life, more hospitalizations and worse prognosis.

Exercise intensity and cardiac disease development in carriers of titin truncating 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 And Results: Truncating variant of titin 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).

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.

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.

Gaps in evidence in the treatment of prevalent patients with pulmonary arterial hypertension at intermediate risk: An expert consensus.

Despite the innovations introduced in the 2022 European Society of Cardiology/European Respiratory Society Guidelines on Pulmonary Hypertension, risk discrimination and management of pulmonary arterial hypertension (PAH) patients at intermediate risk still represents a grey zone. Additionally, clinical evidence derived from currently available studies is limited. This expert panel survey intends to aid physicians in choosing the best therapeutic strategy for patients at intermediate risk despite ongoing oral therapy.

Status and timing of angiotensin receptor-neprilysin inhibitor implementation in patients with heart failure and reduced ejection fraction: Data from the Swedish Heart Failure Registry.

Aims: We explored timing, settings and predictors of angiotensin receptor-neprilysin inhibitor (ARNI) initiation in a large, nationwide cohort of patients with heart failure (HF) with reduced ejection fraction (HFrEF).

Methods And Results: Patients with HFrEF (ejection fraction <40%) registered in the Swedish HF Registry in 2017-2021 and naïve to ARNI were evaluated for timing and location of, and their characteristics at ARNI initiation. ARNI use increased from 8.

Does extracorporeal cardiopulmonary resuscitation improve survival with favorable neurological outcome in out-of-hospital cardiac arrest? A systematic review and meta-analysis.

Purpose: Extracorporeal cardiopulmonary resuscitation (E-CPR) may improve survival with favorable neurological outcome in patients with refractory out-of-hospital cardiac arrest (OHCA). Unfortunately, recent results from randomized controlled trials were inconclusive. We performed a meta-analysis to investigate the impact of E-CPR on neurological outcome compared to conventional cardiopulmonary resuscitation (C-CPR).

Right ventricular phenotyping in incident patients with idiopathic pulmonary arterial hypertension.

Background: Right ventricular (RV) imaging has not a definite role in risk stratification of pulmonary arterial hypertension (PAH) patients. We tested the hypothesis that echocardiography-derived phenotypes, depicting different degrees of RV remodeling and dysfunction, may provide additional prognostic information to current risk stratification tools.

Methods: Consecutive incident PAH patients aged ≥18 years, diagnosed between January 2005 and December 2021, underwent clinical assessment, right heart catheterization, standard echocardiography.