Coronary artery calcium score: we know where we are but not where we may be

Cardiac computed tomography angiography (CCTA) has emerged as a cost-effective and time-saving technique for excluding coronary artery disease. One valuable tool obtained by CCTA is the coronary artery calcium (CAC) score. The use of CAC scoring has shown promise in risk assessment and stratification of cardiovascular disease. CAC scores can be complemented by plaque analysis to assess vulnerable plaque characteristics and further refine risk assessment. This paper aims to provide a comprehensive understanding of the value of the CAC as a prognostic tool and its implications for patient risk assessment, treatment strategies and outcomes. CAC scoring has demonstrated superior ability in stratifying patients, especially asymptomatic individuals, compared to traditional risk factors and scoring systems. The main evidence suggests that individuals with a CAC score of 0 had a good long-term prognosis, while elevated CAC score is associated with increased cardiovascular risk. Finally, the clinical power of CAC scoring and the develop of new models for risk stratification could be enhanced by machine learning algorithms

Natural History of Arrhythmogenic Cardiomyopathy

Arrhythmogenic cardiomyopathy (AC) is a heart muscle disease characterized by a scarred ventricular myocardium with a distinctive propensity to ventricular arrhythmias (VAs) and sudden cardiac death, especially in young athletes. Arrhythmogenic right ventricular cardiomyopathy (ARVC) represents the best characterized variant of AC, with a peculiar genetic background, established diagnostic criteria and management guidelines; however, the identification of nongenetic causes of the disease, combined with the common demonstration of biventricular and left-dominant forms, has led to coin the term of "arrhythmogenic cardiomyopathy", to better define the broad spectrum of the disease phenotypic expressions. The genetic basis of AC are pathogenic mutations in genes encoding the cardiac desmosomes, but also non-desmosomal and nongenetic variants were reported in patients with AC, some of which showing overlapping phenotypes with other non-ischemic diseases. The natural history of AC is characterized by VAs and progressive deterioration of cardiac performance. Different phases of the disease are recognized, each characterized by pathological and clinical features. Arrhythmic manifestations are age-related: Ventricular fibrillation and SCD are more frequent in young people, while sustained ventricular tachycardia is more common in the elderly, depending on the different nature of the myocardial lesions. This review aims to address the genetic basis, the clinical course and the phenotypic variants of AC

Arrhythmogenic Cardiomyopathy and Sports Activity

Arrhythmogenic right-ventricular cardiomyopathy (ARVC) is a genetically determined heart disease characterized by progressive myocyte death and substitution by fibrofatty tissue. Life-threatening ventricular arrhythmias may occur during the course of the disease and are distinctively triggered by sports activity: for this reason, ARVC is one of the leading causes of sudden death in the athlete. Early identification of affected athletes by preparticipation screening in the pre-symptomatic phase is essential, but differential diagnosis with the athlete's heart may be challenging. Variants with predominant involvement of the left ventricle are difficult to diagnose unless cardiac magnetic resonance is performed. Athletes with overt ARVC or asymptomatic carriers of pathological gene mutations, including those with an implantable cardioverter defibrillator, should refrain from competitive sports, while a moderate-intensity recreational physical activity appears safe

Arrhythmogenic Cardiomyopathy\u2014Current Treatment and Future Options

Arrhythmogenic cardiomyopathy (ACM) is an inheritable heart muscle disease characterised pathologically by fibrofatty myocardial replacement and clinically by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Although, in its original description, the disease was believed to predominantly involve the right ventricle, biventricular and left-dominant variants, in which the myocardial lesions affect in parallel or even mostly the left ventricle, are nowadays commonly observed. The clinical management of these patients has two main purposes: the prevention of SCD and the control of arrhythmic and heart failure (HF) events. An implantable cardioverter defibrillator (ICD) is the only proven lifesaving treatment, despite significant morbidity because of device-related complications and inappropriate shocks. Selection of patients who can benefit the most from ICD therapy is one of the most challenging issues in clinical practice. Risk stratification in ACM patients is mostly based on arrhythmic burden and ventricular dysfunction severity, although other clinical features resulting from electrocardiogram and imaging modalities such as cardiac magnetic resonance may have a role. Medical therapy is crucial for treatment of VAs and the prevention of negative ventricular remodelling. In this regard, the efficacy of novel anti-HF molecules and drugs acting on the inflammatory pathway in patients with ACM is, to date, unknown. Catheter ablation represents an effective strategy to treat ventricular tachycardia relapses and recurrent ICD shocks. The present review will address the current strategies for prevention of SCD and treatment of VAs and HF in patients with ACM

The subcutaneous implantable cardioverter-defibrillator in clinical practice

Overcoming lead-related complications, the subcutaneous implantable cardioverter-defibrillator (S-ICD) represents the greatest advancement in the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Due to the absence of leads within the body, the S-ICD requires an innovative approach for sensing of subcutaneous signals and defibrillation. The aim of this review is to analyze the current evidence regarding patient selection, technical aspects such as the implant technique, programming and follow-up, as well as complications and troubleshooting of this innovative technology

Impact of coronavirus disease-19 outbreak on arrhythmic events and mortality among implantable cardioverter defibrillator patients followed up by remote monitoring: a single center study from the Veneto region of Italy

The 2020 severe acute respiratory syndrome coronavirus 2 outbreak entailed reduced availability of traditional (in-office) cardiology consultations. Remote monitoring is an alternative way of caring that may potentially mitigate the negative effects of the epidemic to the care of cardiovascular diseases. We evaluated the outcome of implantable cardioverter defibrillator (ICD) carriers followed up remotely in 2020 (epidemic period) versus 2019 (control)