Pentaspline Pulsed Field Ablation Versus High-Power Short-Duration/Very High-Power Short-Duration Radiofrequency Ablation in Atrial Fibrillation: A Meta-Analysis.

Background: Pulsed field energy has been proposed as alternative to radiofrequency energy in atrial fibrillation (AF) ablation.

Objective: To compare data from studies assessing AF ablation with pulsed field ablation (PFA) versus high-power short-duration (HPSD) or very HPSD (vHPSD) radiofrequency ablation (RFA) in terms of AF recurrence, procedure-related complications, fluoroscopy and procedure times.

Methods: A search of online scientific libraries (from inception to October 1, 2024) was performed.

Hypertrophic Cardiomyopathy and Phenocopies: New Therapies for Old Diseases-Current Evidence and Future Perspectives.

The hypertrophic cardiomyopathy (HCM) clinical phenotype includes sarcomeric HCM, which is the most common form of inherited cardiomyopathy with a population prevalence of 1:500, and phenocopies such as cardiac amyloidosis and Anderson-Fabry disease, which are considered rare diseases. Identification of cardiac and non-cardiac red flags in the context of multi-organ syndrome, multimodality imaging, including echocardiography, cardiac magnetic resonance, and genetic testing, has a central role in the diagnostic pathway. Identifying the specific disease underlying the hypertrophic phenotype is very important since many disease-modifying therapies are currently available, and phase 3 trials for new treatments have been completed or are ongoing.

Atrial cardiomyopathy: new pathophysiological and clinical aspects.

Atrial cardiomyopathy (ACM) is increasingly recognized as a key contributor to the development and perpetuation of atrial fibrillation (AF), a prevalent cardiac arrhythmia with significant clinical implications. ACM involves complex structural, electrical, and functional remodeling of the atrial myocardium, driven by various pathological conditions such as hypertension, heart failure, and obesity. Key mechanisms include atrial fibrosis, inflammation, and oxidative stress, which collectively contribute to the pro-arrhythmic and pro-thrombotic state associated with AF.

[Heart, sport, and genetic testing].

Genetic tests are useful in the, even early, diagnosis and management of various hereditary pathologies with cardiovascular involvement such as cardiomyopathies, arrhythmic diseases, and aortic diseases. The diagnosis of these diseases can be challenging in athletes where the ECG and echocardiogram show physiological alterations due to intense physical activity. However, if performed inappropriately and if not interpreted by a team of professionals with multidisciplinary skills, genetic tests can lead to unnecessary disqualifications from sports activity with important psychological repercussions as well as on the athlete's career.

Subclinical Cardiac Involvement in Asymptomatic ATTR Mutation Carriers: Insights from Cardiac MRI, Myocardial Strain, and Mapping Techniques.

Transthyretin cardiac amyloidosis (ATTR-CA) leads to myocardial infiltration, affecting prognosis and survival. Diagnosing early-stage ATTR-CA remains challenging due to its subtle manifestations. This study investigates subclinical myocardial alterations in asymptomatic ATTR mutation carriers (ATTR-MC) using advanced cardiac magnetic resonance (CMR) techniques, including T1 mapping and myocardial strain analysis.

Change in prevalence of ATTR variants in Italy - Results from a National Survey.

Introduction: Hereditary transthyretin amyloidosis (ATTRv) is a rare, heterogenous, inherited disorder caused by over 130 gene mutations. Its prevalence was estimated to 4.33/million in 2020 in Italy.

ANMCO position paper 'Hypertrophic cardiomyopathy: from diagnosis to treatment'.

Hypertrophic cardiomyopathy (HCM) is a non-rare genetic cardiomyopathy, with an estimated prevalence of 1:500, characterized by an increase in the left ventricular wall thickness in the absence of increased loading conditions. The hypertrophy is mostly asymmetric and predominantly affects the basal septum and anterior wall. Left ventricular outflow tract obstruction, at rest or after provocative tests, is detected in many patients and represents the primary cause of reduced functional capacity, as well as an independent predictor of sudden cardiac death and advanced heart failure.

ANMCO position paper: diagnosis and treatment of heart failure with preserved systolic function.

Heart failure is the leading cardiovascular cause of hospitalization with an increasing prevalence, especially in older patients. About 50% of patients with heart failure have preserved ventricular function, a form of heart failure that, until a few years ago, was orphaned by pharmacological treatments effective in reducing hospitalization and mortality. New trials, which have tested the use of gliflozins in patients with heart failure with preserved ejection fraction (HFpEF), have for the first time demonstrated their effectiveness in changing the natural history of this insidious and frequent form of heart failure.

Aldosterone-Related Cardiovascular Disease and Benefits of Mineralocorticoid Receptor Antagonists in Clinical Practice.

High levels of aldosterone are associated with vascular and cardiac remodeling, myocardial fibrosis, and endothelial dysfunction with consequent increased risk of cardiovascular events and cardiovascular mortality. Indeed, mineralcorticoid receptor antagonists (MRAs) are recommended in the treatment of arterial hypertension, heart failure, alone or associated with chronic kidney disease. Nevertheless, molecular pathways underlying aldosterone-induced cardiac remodeling are poorly investigated.

[ANMCO Position paper: Hypertrophic cardiomyopathy: from diagnosis to treatment].

Hypertrophic cardiomyopathy (HCM) is a non-rare genetic cardiomyopathy, with an estimated prevalence of 1:500, characterized by an increase in the left ventricular wall thickness in absence of increased loading conditions. The hypertrophy is mostly asymmetric and predominantly affects the basal septum and anterior wall. Left ventricular outflow tract obstruction, at rest or after provocative tests, is detected in many patients and represents the primary cause of reduced functional capacity, as well as an independent predictor of sudden cardiac death and advanced heart failure.

[Management of cardiac amyloidosis in Italy: a national survey of the Italian Cardiac Amyloidosis Network (RIAC)].

Background: The national survey of the SIC-ANMCO Amyloidosis Centers was designed to create a geographical mapping of the centers that identify and follow patients with cardiac amyloidosis in Italy and to describe their diagnostic capabilities and multidisciplinary organization.

Methods: The survey was administered to 212 centers in Italy. Among them, 153 centers responded (72%), 31/35 (89%) of which were academic medical centers and 122/177 (69%) hospitals.

Females with Fabry disease: an expert opinion on diagnosis, clinical management, current challenges and unmet needs.

Females with Fabry disease (FD) often have a milder phenotype, later symptom onset, and slower disease progression than males, causing delayed diagnosis and undertreatment. A survey was conducted at nine Italian FD centers to evaluate routine management of females with FD; results were discussed at a meeting of eleven Italian specialists and recommendations developed. Of the 227 females managed by the physicians surveyed, 85% were diagnosed through family screening and 38.

[ANMCO Position paper: Management of heart failure with preserved ejection fraction].

Heart failure is the leading cardiovascular cause of hospitalization with an increasing prevalence, especially in older patients. About 50% of patients with heart failure have preserved ventricular function, a form of heart failure that, until a few years ago, was orphaned by pharmacological treatments effective in reducing hospitalization and mortality. New trials, which have tested the use of gliflozins in patients with heart failure with preserved ejection fraction (HFpEF), have for the first time demonstrated their effectiveness in changing the natural history of this insidious and frequent form of heart failure.

Exploring Cardiac Sympathetic Denervation in Transthyretin-Mediated Hereditary Amyloidosis (ATTRv): Insights from I-mIBG Scintigraphy.

Hereditary transthyretin-mediated amyloidosis (ATTRv) is a rare disease characterized by the deposition of amyloid in the heart and peripheral nerves, particularly affecting small fibers. This study aims to evaluate autonomic cardiac involvement in ATTRv. Twelve patients with ATTRv and twelve sex- and age-matched healthy subjects underwent I-mIBG scintigraphy to evaluate the early and late heart-to-mediastinum ratio (eH/M and lH/M), Tc-HDP bone scan scintigraphy, and neurophysiological assessments.

Fabry Cardiomyopathy: Myocardial Fibrosis, Inflammation, and Down-Regulation of Mannose-6-Phosphate Receptors Cause Low Accessibility to Enzyme Replacement Therapy.

Background: The clinical impact of enzyme replacement therapy on advanced Fabry disease cardiomyopathy appears to be limited. The pathologic mechanisms involved are still unclear.

Methods And Results: Ten male patients with advanced Fabry disease cardiomyopathy on enzyme replacement therapy, whose disease progressed from maximal wall thickness of 15.

SGLT2i reduce arrhythmic events in heart failure patients with cardiac implantable electronic devices.

Background: Sodium glucose cotransporter 2 inhibitors (SGLT2i) represent one of the four pillars of heart failure (HF) pharmacological therapy.

Objective: The study aims to clarify SGLT2i antiarrhythmic effect on patients with HF with reduced ejection fraction (HFrEF) in terms of atrial and ventricular arrhythmias (AAs and VAs) reduction.

Methods: HFrEF carriers of implantable cardioverter defibrillator (ICD) or cardiac resynchronization therapy defibrillator (CRT-D) followed by remote monitoring of Policlinico Umberto I of Rome for 1 year before and after SGLT2i therapy initiation were enrolled in the study.

Anderson-Fabry Disease: Focus on Ophthalmological Implications.

Fabry disease (FD) is a rare X-linked lysosomal storage disorder with a broad spectrum of clinical manifestations, including severe complications, such as end-stage renal disease, hypertrophic cardiomyopathy, and cerebrovascular disease. Enzyme replacement therapy (ERT), when initiated early, has been shown to reduce the incidence of severe events and slow disease progression. In the classic form, characterized by the absence of α-galactosidase A (α-Gal A) enzyme activity, diagnosis is straightforward.

Cardiac contractility modulation: from molecular patterns to tailored treatment in heart failure subgroups.

Cardiac contractility modulation (CCM) signals are non-excitatory signals that are applied during the myocyte's absolute refractory period. These signals have been demonstrated to have an inotropic effect without increasing myocardial oxygen consumption. This has been observed in both preclinical animal studies and randomized clinical trials.

Thromboembolic Risk in Sinus Rhythm: A New Paradigm in Light Chain Cardiac Amyloidosis.

Thromboembolic risk is increased in cardiac amyloidosis, and this goes beyond the occurrence of atrial fibrillation in these patients. A 56-year-old man was admitted to our hospital for a presyncopal episode. Clinical and instrumental findings led to a diagnosis of light chain cardiac amyloidosis.

Efficacy of Modern Therapies for Heart Failure with Reduced Ejection Fraction in Specific Population Subgroups: A Systematic Review and Network Meta-Analysis.

Introduction: The efficacy and safety of emerging therapies for heart failure with reduced ejection fraction (HFrEF) have never been compared in specific subgroups of patients.

Methods: PubMed, Cochrane Registry, Web of Science, Scopus, and EMBASE libraries were used to extract data. We used the following keywords: (heart failure with reduced ejection fraction OR HFrEF) AND (treatment OR therapy) OR (cardiovascular death) OR (hospitalization for heart failure).

Oxidative Stress in Transthyretin-Mediated Amyloidosis: An Exploratory Study.

Transthyretin-mediated amyloidosis (ATTR) is a systemic disease with protein precipitation in many tissues, mainly the peripheral nerve and heart. Both genetic (ATTRv, "v" for variant) and wild-type (ATTRwt) forms are known. Beyond the steric encumbrance, precipitated transthyretin seems to have a toxic effect.

Usefulness of empiric superior vena cava isolation in paroxysmal atrial fibrillation ablation: a meta-analysis of randomized clinical trials.

Background: The long-term success rate of pulmonary vein isolation (PVI) is suboptimal due to the presence of non-pulmonary vein (PV) foci that can trigger atrial fibrillation (AF) in up to 11%. Among non-PV triggers, the superior vena cava (SVC) is a major site of origin of ectopic beats initiating AF.

Objective: To compare data from randomized controlled trials (RCTs) assessing PVI + empiric SVC isolation (SVCI) versus PVI alone in terms of AF recurrence, procedure-related complications, and fluoroscopic and procedural times.

Immunomodulating and Immunosuppressive Therapy for Virus-Negative Immune-Mediated Myocarditis.

Myocarditis is an inflammatory disease of the myocardium caused by infectious and noninfectious agents. Clinical manifestations range from mildly symptomatic forms to acute heart failure, cardiogenic shock, life-threatening arrhythmias and sudden death. Myocarditis is still a challenging diagnosis because of its wide variability in clinical presentation and unpredictable course.