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Arrhythmogenic cardiomyopathy (CMA) is a cardiac disease characterized by non-ischemic ventricular scarring and electrical instability. The diagnosis of CMA still remains challenging today and requires the use of a set of criteria, since no single diagnostic test represents the gold standard. The first diagnostic criteria were defined and disseminated in 1994 and then revised in 2010, focusing mainly on right ventricular involvement. In 2019, an international panel of experts identified the limitations of the previous diagnostic criteria. The 2020 Padua criteria included a specific pathway for the diagnosis of left ventricular variants and emphasized the need for the use of cardiac magnetic resonance imaging in the characterization of myocardial scarring. These criteria were further refined and published in 2023 as European Task Force (TF) criteria, thus gaining international recognition.Exploring the history of CMA and its diagnosis, in this review we analyze the changes and progress in the 20 years that have occurred from the first version of the criteria in 1994 to the latest in European TF of 2023, highlighting the evolution of our knowledge of the pathobiology and morpho-functional characteristics of the disease. One of the most relevant updates is undoubtedly the introduction of the concept of "scarring/arrhythmogenic cardiomyopathy", a definition that enhances the main features of the pathology and emphasizes the multiplicity of phenotypes and clinical presentations independent of etiology.

Chronic kidney disease and cardiovascular disease are strictly connected each other with a bidirectional interaction. Thus, the prevention of cardio-renal damage, as its appropriate treatment, are essential steps for a correct management of long-term patients' prognosis. Several preventive and therapeutic strategies, pharmacological and not, are now available for cardio-renal damage prevention and treatment, and for the management of its complications. The second part of this consensus document focuses on the management and treatment of cardio-renal damage, directing the attention on the correct use of drugs that may slow renal disease progression, on the application of preventive strategies in case of invasive cardiac procedures with the use of contrast agents, and on the accurate use of cardiological drugs in patients with chronic kidney disease.

Chronic kidney disease (CKD) and cardiovascular (CV) disease are highly prevalent conditions in the general population and are strictly connected to each other with a bidirectional interaction. In patients affected by CKD, the leading cause of morbidity and mortality is represented by CV disease, since CKD promotes the atherosclerotic process increasing inflammation, and modifying lipid and bone mineral metabolism. On the other side, a strict relationship exists between CKD and CV risk factors, which are prevalent in nephropathic patients and impose a stringent assessment of the risk of CV events in this population together with an optimized pharmacological approach, complicated by the coexistence of the two pathological conditions. The first part of this consensus document focuses on the mechanisms of cardio-renal damage and on the impact, as well as the management, of the main CV risk factors in the context of CKD.

Although the clinical course of COVID-19 in its acute phase is now delineated, less known is its late phase characterized by a heterogeneous series of sequelae affecting various organs and systems, including the cardiovascular system, which continue after the acute episode or arise after their resolution. This syndrome, now referred with the new acronym "PASC" (post-acute sequelae of SARS-CoV-2 infection) has been formally recognized by various scientific societies and international organizations that have proposed various definitions. The World Health Organization defines PASC, distinguishing it from "ongoing symptomatic COVID-19", as a condition that arises few weeks after infection, persists at least 8 weeks, and cannot be explained by alternative diagnoses.There are multiple mechanisms responsible for PASC: inflammation, immune activation, viral persistence, activation of latent viruses, endothelial dysfunction, impaired response to exercise, and profound cardiac deconditioning following viral infection. The key symptoms of PASC are palpitations, effort dyspnea, chest pain, exercise intolerance, and postural orthostatic tachycardia syndrome.For PASC treatment, it may be useful to take salt and fluid loading, to reduce symptoms such as tachycardia, palpitations, and/or orthostatic hypotension, or in some subjects the use of drugs such as beta-blockers, non-dihydropyridine calcium channel blockers, ivabradine, and fludrocortisone.Finally, in PASC a gradual resumption of physical activity is recommended, starting with recumbent or semi-recumbent exercise, such as cycling, swimming, or rowing, and then moving on to exercise in an upright position such as running when the ability to stand improves without dyspnea appearance. Exercise duration should also be short initially (5 to 10 min per day), with gradual increases as functional capacity improves.

Vaccine-associated myocarditis and pericarditis usually develop within 14 days of COVID-19 vaccination, are exceptionally rare, manifest with mild clinical pictures and are commonly characterized by a favorable evolution. Young men inoculated with two doses of an mRNA vaccine are the subgroup at higher risk. Recent epidemiological studies evaluated the incidence and risk of vaccine-associated myocarditis and pericarditis among men and women, in different ranges of age and specific types of vaccines. Long-term population analyses demonstrated that the cardiovascular risk conferred by COVID-19 extends beyond the acute phase, representing the rationale for implementing prevention strategies for SARS-CoV-2 infection, monitoring specific populations at higher risk and pursuing the completion of the vaccination campaign. This document provides an update on the most recent scientific evidence and critical interpretation of available data in constant evolution towards personalized strategies of immunization.

The use of sacubitril/valsartan has been fully recognized in the most recent European and American guidelines that recommend in class I the prescription of this drug in heart failure patients with reduced systolic function. Besides the effects on cardiovascular mortality and heart failure hospitalization, sacubitril/valsartan significantly reduces NT-proBNP levels and improves cardiac remodeling, recognized as one of the mechanistic effects of the drug that is linked to favorable prognostic effects. A careful evaluation of the patients' clinical profile is needed to implement the use of sacubitril/valsartan into clinical practice and to make the treatment successful. This second part of the position paper focuses on the mechanistic effects of angiotensin receptor-neprilysin inhibitors and on its placement in current guidelines, also suggesting the use of sacubitril/valsartan in specific clinical settings.

Renin-angiotensin-aldosterone (RAAS) system inhibition is a mainstay of the pharmacological treatment of heart failure with reduced ejection fraction and has been implemented by the introduction of angiotensin receptor-neprilysin inhibitors (ARNI), that combine RAAS inhibition with the inhibition of neprilysin, enhancing the favorable effects of natriuretic peptides. The PARADIGM-HF trial demonstrated a favorable effect of sacubitril/valsartan over enalapril in terms of mortality and heart failure hospitalization rate reduction. Then several randomized clinical trials and observational studies confirmed the favorable role of ARNI in different clinical scenarios, supporting the guideline class I recommendation for the use of sacubitril/valsartan in patients with reduced systolic function. The first part of this position paper summarizes the history of RAAS inhibition and reports the results of ARNI trials that support the recommendations of the most recent guidelines.

Atrial fibrillation (AF) is the most common arrhythmia worldwide. Thromboembolism from the left atrial appendage (LAA) is the most feared complication in patients with AF. The cornerstone for the management of AF is oral anticoagulation to reduce the incidence of cardioembolic stroke. There is, however, a significant proportion of patients who cannot undergo long-term oral anticoagulation. Transcatheter LAA occlusion is an evolving technology with proven benefits in terms of AF-related stroke prevention, representing a valid alternative to anticoagulation for high-risk patients with contraindications for long-term oral anticoagulation. This has resulted in the development of a plethora of transcatheter devices to achieve endocardial occlusion or epicardial exclusion of the LAA.A panel of expert Italian cardiologists gathered under the aegis of the Italian Society of Interventional Cardiology (SICI-GISE) with the aim of reviewing the most relevant aspects of LAA occlusion, underlying anatomy and pathophysiology, summarizing current clinical knowledge, and discussing the practicalities of available devices and imaging techniques. Finally, the position paper highlights the importance of an adequate environment and of an appropriate organization in order to optimize all steps of the procedure.

The current COVID-19 pandemic has renewed interest in providing healthcare services based on the implementation of innovative technologies. Such strategy capillarizes the therapeutic opportunities for larger urban areas, mostly when people are living under extraordinarily difficult circumstances. Improving care delivery in cardiovascular diseases appears particularly feasible when telemedicine is pursued, especially with regard to baseline standard 12-lead electrocardiography, ambulatory electrocardiographic monitoring, and 24-hour ambulatory blood pressure monitoring. Nowadays, these first-line cardiovascular examinations are also available in health centers and pharmacies, and in recent months, there has been an increasing demand of such local services in the absence of specific rules and regulations regarding technical requirements and standards of interpretation that ensure a high quality clinical consultation.The purpose of this position paper is to provide critical requirements for the type/model of devices to be used, training dedicated to healthcare personnel, ensuring security of sensitive data, highlighting type of platforms to be used, as well as for maintaining high reporting quality and standards.

The coronavirus disease (COVID-19) pandemic has caused 2.69 million deaths and 122 million infections. Great efforts have been made worldwide to promptly develop effective vaccines and reduce morbidity and mortality rates from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Available vaccines have proven highly effective at preventing symptomatic disease in clinical trials and real-world reports and are playing an essential role in flattening the epidemiology curve and, mostly, in reducing COVID-19 hospitalizations. Some concerns have been raised after very rare cases of myocarditis and pericarditis recently reported by the Centers for Disease Control and Prevention (CDC) as potentially associated with COVID-19 mRNA vaccinations, namely the Pfizer-BioNTech mRNA vaccine (BNT162b2) and the Moderna mRNA vaccine (mRNA-1273). Therefore, the aim of this document is to explore the possible link between COVID-19 mRNA vaccination and the development of myocarditis and/or pericarditis by performing a critical analysis of available data and to provide indications for specific subgroups of individuals.

In over a year, the COVID-19 pandemic caused 2.69 million deaths and 122 million infections. Social isolation and distancing measures have been the only prevention available for months. Scientific research has done a great deal of work, developing in a few months safe and effective vaccines against COVID-19. In the European Union, nowadays, four vaccines have been authorized for use: Pfizer-BioNTech, Moderna, ChAdOx1 (AstraZeneca/Oxford), Janssen (Johnson & Johnson), and three others are currently under rolling review.Vaccine allocation policy is crucial to optimize the advantage of treatment preferring people with the highest risk of contagion. These days the priority in the vaccination program is of particular importance since it has become clear that the number of vaccines is not sufficient for the entire Italian population in the short term. Cardiovascular diseases are frequently associated with severe COVID-19 infections, leading to the worst prognosis. The elderly population suffering from cardiovascular diseases is, therefore, to be considered a particularly vulnerable population. However, age cannot be considered the only discriminating factor because in the young-adult population suffering from severe forms of heart disease, the prognosis, if affected by COVID-19, is particularly ominous and these patients should have priority access to the vaccination program. The aim of this position paper is to establish a consensus on a priority in the vaccination of COVID-19 among subjects suffering from different cardiovascular diseases.

Myocarditis is an inflammatory heart disease that can occur acutely, as in acute myocarditis, or persistently, as in chronic myocarditis or chronic inflammatory cardiomyopathy. Different agents can induce myocarditis, with viruses being the most common triggers. Generally, acute myocarditis affects relatively young people and men more than women. Myocarditis has a broad spectrum of clinical presentations and evolution trajectories, although most cases resolve spontaneously. Patients with reduced left ventricular ejection fraction, heart failure symptoms, advanced atrioventricular block, sustained ventricular arrhythmias or cardiogenic shock (the latter known as fulminant myocarditis) are at increased risk for death and heart transplantation. The presentation of chronic inflammatory cardiomyopathy may be more subtle, with progressive symptoms of heart failure or appearance of rhythm disturbance, not rarely preceded by an infective episode. Autoimmune disorder or systemic inflammatory conditions can be another significant predisposing substrate of myocarditis, especially in women. Emerging causes of myocarditis are drug-related like the new anticancer therapies, the immune checkpoint inhibitors. In this Italian Association of Hospital Cardiologists (ANMCO) and Italian Society of Cardiology (SIC) expert consensus document on myocarditis, we propose diagnostic strategies for identifying possible causes of the disease and factors associated with increased risk. Finally, we propose potential treatments and when referring patients to tertiary centers, especially for high-risk patients. Even if endomyocardial biopsy is the invasive diagnostic tool for making definitive diagnosis and differentiation of histological subtypes (i.e., lymphocytic vs eosinophilic vs giant cell myocarditis), it is not always readily available in all centers. Thus, we propose when this exam is mandatory or when it can be postponed or substituted by cardiac magnetic resonance imaging. This document reflects the Italian perspective on managing patients with myocarditis and their follow-up, considering also current US and European scientific position statements.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease that predisposes to the occurrence of ventricular arrhythmias and sudden death, particularly in the young and athlete. The classic variant of the disease predominantly affects the right ventricle, but phenotypic variants with early and prevalent left ventricular involvement ("left-dominant" ARVC) have also been described, supporting the concept that arrhythmogenic cardiomyopathy is a disease of both ventricles. The diagnosis is multiparametric and is based on a series of criteria, including ECG abnormalities, arrhythmic manifestations, morpho-functional abnormalities and genetic defects. The main goal of therapy is sudden death prevention. Implant of a cardioverter-defibrillator is the most effective strategy for prevention of sudden death, but it should be reserved to selected patients after accurate risk stratification, in view of the high complication rate over a long-term follow-up, the costs and the significant psychological impact of such therapy, especially in the young individual. The other therapies (either pharmacological or not) are palliative and aimed at relieving symptoms and preventing disease progression. The definitive cure of ARVC will be based on the discovery of the molecular mechanisms that are involved in the etiology and pathogenesis of the disease.

Multimodal imaging plays a pivotal role in the assessment of the thoracic aorta, both in chronic and acute settings. Moving from improved knowledge on the structure and function of the aortic wall, as well as on its pathophysiology and histopathology, appropriate utilization of each imaging modality results into a better definition of the patient's need and proper treatment strategy. This review is aimed at highlighting the most critical aspects in this field, providing cardiologists with some novel clues for the imaging approach to patients with thoracic aortic disease.

Improved knowledge of the structure and function of the aortic wall, as well as its pathophysiology and histopathology, will result in an increasing impact on clinical evaluation and treatment of thoracic aortic diseases. This opinion paper highlights the main "paradigmatic shifts" in this field, providing cardiologists with some novel clues for the clinical approach to patients with thoracic aortic disease.

Although the incidence of sudden cardiac death (SCD) is greater in men than in women, it represents an important mode of death also in the female gender. Sex-related differences have been identified not only in the prevalence of the phenomenon, but also in risk factors and etiology of SCD. The peripartum period represents a peculiar trigger of SCD in women with underlying cardiovascular substrates. Several lesions, both congenital and acquired, may be identified at postmortem in SCD victims, including coronary artery, myocardial, valve, and aortic diseases. While the incidence of SCD increases progressively with age in adult elderly women to reach a 1:1 male:female ratio after the age of 80 years, mostly due to the increasing incidence of atherosclerotic disease in the postmenopausal period, SCD in young women is a rare event and usually associated with non-atherosclerotic disease, such as mitral valve prolapse, spontaneous coronary dissection, myocarditis, inherited cardiomyopathies and congenital heart diseases. The heart can be found structurally normal and inherited ion channel diseases are often implicated. Gender differences in the risk of SCD deserve further attention, since they affect the evaluation of interventions designed to reduce the rate of female SCD.

Bicuspid aortic valve (BAV) is the most common congenital heart disease, whose natural history is characterized by the incidence of clinically relevant valvular (stenosis, regurgitation, endocarditis) and/or vascular complications regarding the thoracic aorta (dilation, aneurysm, dissection) and, rarely, intracranial and epiaortic arteries. BAV may be heritable, with an autosomal dominant pattern of inheritance with reduced penetrance; moreover, some data suggest that BAV and thoracic aorta aneurysm are independent manifestations of a single gene defect. The prevalence of BAV and its susceptibility to valvular and aortic complications during the whole life result into the need of strict clinical follow-up and appropriate therapies (medical as well as surgical) to be addressed according to guidelines specifically designed for these patients.

Both the American Heart Association and the European Society of Cardiology consensus panel recommendations agree that cardiovascular screening for young competitive athletes is justifiable and compelling on ethical, legal, and medical grounds. However, there is a considerable discordance in the consensus guidelines on the pre-participation screening protocols used among European and US cardiologists/sports medicine physicians, with and without 12-lead electrocardiogram (ECG). In 1982 a nationwide program of pre-participation screening including ECG was launched in Italy. The aim of this article is to examine whether this long-running screening program should be considered a valid and advisable public health strategy. The analysis of data coming from the Italian experience indicates that ECG screening has provided adequate sensitivity and specificity for detection of potentially lethal cardiomyopathy or arrhythmias and has led to substantial reduction of mortality of young competitive athletes by approximately 90%. Screening has been feasible thanks to the Italian Health System, which is developed in terms of healthcare and prevention services, and because of the limited costs of cardiovascular evaluation in the setting of a mass program. The available scientific evidence suggests to adopt a screening protocol including ECG, which is the only screening tool proved to be effective. This article will also address the available data and criticisms concerning the screening program to prevent sudden cardiac death of middle-aged/senior athletes engaged in leisure-time sports activity.

Although sudden cardiac death is one of the most important mode of death in Western Countries, pathologists and public health physicians have not given this problem the attention it deserves. New methods of preventing potentially fatal arrhythmias have been developed and the accurate diagnosis of the causes of sudden cardiac death is now of particular importance. Pathologists are responsible for determining the precise cause of sudden death but there is considerable variation in the way in which they approach this increasingly complex task. The Association for European Cardiovascular Pathology developed these Guidelines, which represent the minimum standard that is required in the routine autopsy practice for the adequate assessment of sudden cardiac death, including not only a protocol for heart examination and histological sampling, but also for toxicology and molecular investigation. Our recommendations apply to University Medical Centres, Regional and District Hospitals and all types of Forensic Medicine Institutes. If a uniform method of investigation is adopted throughout the European Union, this will lead to improvements in standards of practice, allow meaningful comparisons between different communities and regions and, most importantly, permit future trends in the patterns of disease causing sudden death to be monitored.

The Italian Scientific Associations of Cardiologists and Cardiovascular Pathologists have produced this consensus document on the diagnostic role of endomyocardial biopsy (EMB) in terms of techniques, analysis and reporting. The document is intended for clinical cardiologists, hemodynamic experts, electrophysiologists, surgical pathologists, and cardiac surgeons. It has three main aims: a) to collocate EMB in the context of currently available tools for diagnosis of heart diseases; b) to provide recommendations for rational implementation; c) to outline key characteristics (standards) for Italian cardiology and surgical pathology centers that perform and analyze EMB. A general lack of prospective, controlled studies addressing EMB prohibited the use of traditional evidence-based recommendations that rely on classes of available evidence. Thus, it was agreed that three key points should be taken into account: a) the specific pathology to be diagnosed (or excluded); b) the existence of any alternative, non-invasive diagnostic techniques; c) the overall consequences of reaching a definite diagnosis on patients' clinical management. Accordingly, we propose recommendations for EMB based on the following levels of diagnostic value: level 1: no alternative method exists to reach a definite diagnosis that can have obvious consequences for clinical management; level 2a: no alternative method exists to reach a definite diagnosis; however, the implications for clinical management are uncertain; level 2b: no alternative method exists to reach a definite diagnosis; however, the diagnosis would not influence clinical management; level 3: an alternative method exists to reach a definite diagnosis. The second part of the document proposes current protocols for the preparation, analysis and reporting of EMB in the context of each main pathologic entity. Particular attention is given to tissue characterization and implementation of molecular tests.

Juvenile sudden death and sudden infant death syndrome exert a deep social impact, due to the young age of the victims and the unexpected occurrence of death. Recently, genetically determined ion channel diseases have been demonstrated to account for many forms of juvenile sudden death sine materia and also for some cases of sudden infant death syndrome (Brugada syndrome, long QT and short QT syndromes and catecholaminergic polymorphic ventricular tachycardia). Moreover, a not negligible amount of juvenile sudden deaths are due to myocarditis as a consequence of cardiotropic viruses. Thus, it is now becoming mandatory to apply molecular pathology techniques also to the post mortem study of sudden death. In general, a long interval between death and post mortem exam and inadequate tissue sampling and preservation may increase the poor results of molecular investigation. The aim of this review was to provide evidence of the need to develop a molecular pathology investigation protocol to be used at post mortem.

A 32-year-old man without risk factors for coronary artery disease but moderate cigarette smoking died suddenly 5 years after a cardiological check-up for chest pain on effort. Autopsy investigation ruled out extracardiac causes of death and pointed to a multivessel coronary artery disease with organized and recanalized occlusive thrombosis, sometimes superimposed to smooth muscle cells proliferation, in the absence of peripheral vascular disease. Thromboangiitis obliterans of the coronary arteries or accelerated atherosclerosis complicated by thrombosis due to endothelial erosion are debated as possible etiopathogenetic mechanisms. The case is still open for achieving a final diagnosis.

Balloon coronary angioplasty revascularization is accomplished through a remodelling of the atherosclerotic stenotic wall. Increase in lumen diameter is achieved by plaque cracking with intimal dissection, plaque compression and medial stretching. Acute complications are frequent (3-8%) and mostly consist of occlusive thrombosis occurring upon fissuring of atheromasic plaque, intimal flap with invagination, medio-adventitial dissection in the case of tearing of the tunica media, and atheromatous embolism. Healing process may be so exuberant as to lead to restenosis within a few months.

The coronary arteries from the hearts of 7 patients, 6 male and 1 female, age ranging from 39 to 69 years (mean 56), who died early or in the mid term from percutaneous transluminal coronary angioplasty (PTCA), were investigated by histology, immunohistochemistry and electron microscopy. Atherosclerotic coronary artery disease was present in all: single vessel disease in 2 and double vessel disease in 5. Indication to PTCA was stable angina in 1 patient, unstable angina in 2, postinfarction angina in 2 and acute myocardial infarction in the remaining 2. Thrombolysis was an associated procedure in 3 cases. Time interval between PTCA and death was less than 48 hours in 6, and 4 months in 1. Cause of death was myocardial infarction due to coronary occlusion complicating PTCA in 3 patients, and myocardial infarctions preceding PTCA in 4 patients. 9 coronary segments underwent dilatation: 5 in the anterior descending coronary artery, 3 in the right coronary artery and 1 in the left circumflex artery. Angiographically, the PTCA had been effective (decrease of stenosis > or = 40%) in 5 and failed in 4. Basically, the atherosclerotic plaques were eccentric in 3 and concentric in 6; the histotype was fibrotic in 4, atheromatous in 4 and fibro-atheromatous in 1. Only one case did not show any complication and the dilatation was effective as a consequence of plaque compression and stretching of the underlying tunica media. Complications observed in the other cases with early death consisted of: a) plaque cracks with intimal flaps and hematomas in all 6; b) laceration of the tunica media, even of the disease-free wall, with dissecting hematoma in 2; c) occlusive thrombosis in 3 and d) atheroembolism in 1. The 4 coronary segments, which appeared occluded angiographically soon after PTCA, were clinically interpreted as dissection in 3 and thrombosis in 1, whereas at histology the closing mechanism was an intimal hematoma with flap in 2, occlusive thrombosis in 1, and medio-adventitial dissecting hematoma with thrombosis in 1. Only ruptures of atheromatous plaques were complicated by thrombosis. The patient who died at 4 months exhibited a pattern of restenosis due to intimal smooth muscle cell proliferation. In conclusion, our postmortem study shows that PTCA may be associated with severe damage of the coronary artery consisting of plaque crack and laceration even of the disease-free wall, intimal and medial hematomas, thrombosis and atheroembolism.(ABSTRACT TRUNCATED AT 400 WORDS)

Clinicopathologic findings in two young adults, who complained of ischemic cardiac arrest by ventricular fibrillation and of myocardial infarction complicated by cardiogenic shock respectively, are described. At coronary angiography, coronary arteries appeared normal. In both cases, detailed pathologic examination of the coronary arteries disclosed a focal eccentric atherosclerotic plaque in the proximal descending coronary artery, where selective coronary angiography had failed to reveal filling defects. These observations suggest a more critical attitude in evaluating angiographically normal coronary arteries in patients with myocardial infarction or aborted sudden death, and underline the possibility of arterial lumen stenosis underestimation, especially in the presence of eccentric plaque, with likely compensatory ectasia of the plaque-free wall segment.