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This article summarizes the main electrocardiogram (ECG) findings in dilated cardiomyopathy (DCM) patients. Recent reports are described in the great ‘pot’ of DCM peculiar ECG patterns that are typical of specific forms of DCM. Patients with late gadolinium enhancement on CMR, who are at greatest arrhythmic risk, have also distinctive ECG features. Future studies in large DCM populations should evaluate the diagnostic and prognostic value of the ECG.

Fabry disease (FD) is a rare X-linked inherited lysosomal storage disorder caused by deficient a-galactosidase A activity that leads to an accumulation of glycolipids, mainly globotriaosylceramide (Gb3) and globotriaosylsphingosine, in affected tissues, including the heart. Cardiovascular involvement usually manifests as left ventricular hypertrophy (LVH), myocardial fibrosis, heart failure, and arrhythmias, which limit the quality of life and represent the most common causes of death. Following the introduction of enzyme replacement therapy, early diagnosis and treatment have become essential in slowing down the disease progression and preventing major cardiac complications. Recent advances in the understanding of FD pathophysiology suggest that in addition to Gb3 accumulation, other mechanisms contribute to the development of cardiac damage. FD cardiomyopathy is characterized by an earlier stage of glycosphingolipid accumulation and a later one of hypertrophy. Morphological and functional aspects are not specific in the echocardiographic evaluation of Anderson–Fabry disease. Cardiac magnetic resonance with tissue characterization capability is an accurate technique for the differential diagnosis of LVH. Progress in imaging techniques has improved the diagnosis and staging of FD-related cardiac disease: a decreased myocardial T1 value is specific of FD. Late gadolinium enhancement is typical of the later stage of cardiac involvement but as in other cardiomyopathy is also valuable to predict the outcome and cardiac response to therapy.

Criteria for diagnosis of arrhythmogenic cardiomyopathy (ACM) were first proposed in 1994 and subsequently revised in 2010 and in 2020 by an international task force. According to the last consensus of 2020, ACM is defined as a heart muscle disease affecting right ventricle, left ventricle or both, whose principal pathologic feature is fibrofatty myocardial replacement that impairs systolic ventricular function and predisposes to lethal ventricular arrhythmias. ECG findings not only could help to early recognize affected patients but also could identify the ones with maximum risk of ventricular arrhythmias and sudden cardiac death.

Heart failure (HF) is a major and still growing medical problem and is characterized by episodes of acute decompensation that are associated with a negative prognosis and a significant burden on the patients, doctors, and healthcare resources. Early detection of incipient HF may allow outpatient treatment before patients severely decompensate, thus reducing HF hospitalizations and related costs. The HeartLogic™ algorithm is an automatic, remotely managed system combining data directly related to HF pathophysiology into a single score, the HeartLogic™ index. This index proved to be effective in predicting the risk of incipient HF decompensation, allowing to redistribute resources from low-risk to high-risk patients in a timely and cost-saving manner. The alert-based remote management system seems more efficient than the one based on scheduled remote transmission in terms of caregivers’ workload and alert detection timing. The widespread application of the HeartLogic™ algorithm requires the resolution of logistical and financial issues and the adoption of a pre-defined, functional workflow. In this paper, we reviewed general aspects of remote monitoring in HF patients, the functioning and pathophysiological basis of the HeartLogic index, its efficiency in the management of HF patients, and the economic effects and the organizational revolution associated with its use.

Sudden cardiac death (SCD) can be caused by several clinical conditions, overt or misconceived, which recognize different pathophysiologies determining the development of fatal arrhythmic events. In the various forms of structural heart disease such as ischaemic heart disease, cardiomyopathies (e.g. hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic cardiomyopathy), channelopathies (e.g. long-QT syndrome, congenital short QT, Brugada syndrome, early repolarization (ER) syndrome, and idiopathic ventricular fibrillation) but also in the apparently healthy subject, the 12-lead electrocardiogram (ECG) has proved, over the years, to be a reliable and readily available method for stratifying the risk of adverse arrhythmic events and consequently SCD. Several electrocardiographic markers have been shown to be associated with adverse outcomes in different types of patients. Although with different sensitivity and specificity in each clinical condition, depolarization abnormalities, such as QRS fragmentation, Q waves, QRS duration, left posterior fascicular block, low QRS voltage, and left ventricular hypertrophy and similarly repolarization abnormalities as ER pattern, T wave alternans, QT interval, and QT dispersion, have shown significant efficacy in predicting SCD. Despite the advancement of techniques especially in the field of imaging, the correct interpretation of the 12-lead ECG remains, therefore, an effective tool for assessing the possible prognostic outcome in terms of arrhythmic risk and SCD in different types of patients.

COVID pandemic emergency has forced changes from traditional in-person visits to application of telemedicine in order to overcome the barriers and to deliver care. COVID-19 has accelerated adoption of digital health. During this time, the distance is itself a prevention tool and the use of technology to deliver healthcare services and information has driven the discovery of mobile and connected health services. Health services should to be prepared to integrate the old model of remote monitoring of CIEDs and adopt new digital tools such as mobile Apps and connected sensors.

Aims Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an electrical genetic disease characterized by induction of malignant ventricular arrhythmias during adrenergic stress in structurally normal hearts. CPVT is correlated to syncope or sudden cardiac death (SCD). Usually, it is caused by an autosomal dominant mutation in the cardiac ryanodine receptor (RyR2), an essential gene for Ca2+ homeostasis. Methods and results Our case series refers to: a man (59 years) who came to our attention for a clinical check-up 4 years after implanting bicameral pacemaker at the age of 55 years for complete AV block; and his three sons (E. female 27 years; D. male 25 years; and B. female 17 years) who had evidence of polymorphic non-sustained ventricular tachycardia (NSVT) with increasing effort during stress test. The three sons performed cardiac MRI and underwent genetic test. All three were found to be carriers of the same microdeletion of the RYR 2 gene (1q43- extended for about 49 kb) at the genetic test. They also have non-compacted myocardium at cardiac MRI. The father was also found to be a carrier of the same genetic microdeletion, while the mother was negative to the genetic test. The man was diagnosed to be a carrier of the mutation 4 years after pacemaker implantation. Conclusions Mutation of the RyR2 may have different phenotypic expressions and can be correlated to various clinical manifestations. CPVT is the most common one, and its prompt identification is crucial to prevent subjects from sport-related risks and to plan an efficient therapy. Our case series provides evidence for a careful consideration of such a genetic disorder even in presence of a major AV conduction disease in a relatively young subject. In the present case series, no major adverse events occurred. However, we can, in the aftermath, speculate that if a genetic disorder had been suspected when AV block occurred, a timely diagnosis could have been made earlier also for the sons.