What's New in Arrhythmogenic Cardiomyopathies.

Arrhythmogenic Cardiomyopathy (ACM) is a hereditary cardiomyopathy often presenting with sudden cardiac death (SCD) in young athletic individuals [...]

Stereotactic arrhythmia radioablation: competitor or adjunct to catheter ablation?

Cardiology and Radiation Oncology working together—a new ‘STAR’ on the horizon? Until recently, most cardiologists associated radiation exposure to the heart with potential adverse effects, such as pericarditis, late coronary artery disease or potential damage to cardiac implantable devices. The landmark publication of 2017 reporting a case series of just five patients with recurrent ventricular tachycardia (VT) treated with stereotactic arrhythmia radioablation (STAR) changed this perception and introduced a new area for both cardiac electrophysiology and radiation oncology

Epsilon Waves: The Gate to Understand Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic right ventricular dysplasia (ARVD), first recognized in 1977, is an inherited cardiomyopathy mostly due to mutations in both desmosomal and non-desmosomal genes. ARVD is considered as a leading cause of sudden cardiac death in the young and the athlete. It is characterized by an abnormality in the development of the right ventricular (RV) musculature. The final diagnosis of ARVD was pathologically based on the findings characterized by fibro-fatty infiltration and cardiomyocyte loss predominantly affecting the RV. Epsilon waves are a feature of ARVD reflecting postexcitation of the myocytes in the RV that are interspersed between fibrous and fatty tissue. Epsilon waves are considered to be one of the major diagnostic criteria of ARVD and appear to correlate with the extent of ARVD and arrhythmic risk. In this review, we will briefly review the discovery of ARVD and Epsilon waves, discuss the electrogenesis and various methods for recording Epsilon waves, provide evidence to assist in understanding the pathological and functional changes of the heart in ARVD, thus promoting the management of this disease in patients and family members

Fragmented endocardial signals and early afterdepolarizations during torsades de pointes tachycardia

Background: Bradycardia-induced torsade de pointes (TdP) tachycardia in patients with spontaneous high-degree atrioventricular block (AVB) is common. The aim of this study was to analyze endocardial recordings during TdP in spontaneous high-degree AVB in humans to better understand the electrophysiological mechanisms underlying this phenomenon. Methods: The study group consisted of 5 patients with typical episodes of TdP during spontaneous high-degree AVB. A standard (USCI) temporary bipolar endocardial catheter positioned at the apex of the right ventricle (RV) and bipolar chest leads from two precordial leads V1 and V4 were used to record the tracings during TdP. Results: The presence of a wide spectrum of fragmentations was noted on endocardial electrograms (EGMs), which were invisible on the surface electrocardiogram (ECG) tracing. Endocardial signals indicated that TdP started in the proximity of the RV apex, since the local EGM began prior to the QRS complex on the surface ECG. Early afterdepolarizations (EADs) were observed in 2 out of 5 cases confirming a common opinion about the mechanism of TdP. However, this phenomenon was not observed in 3 other patients suggesting that the arrhythmia was the result of a different mechanism originating in proximity to the RV apex. Conclusions: This work demonstrated early endocardial signals in the RV apex during TdP associated with high-degree AVB in humans, and exhibits a spectrum of fragmented signals in this area occurring on a single or multiple beats. These fragmentations indicate areas of poor conduction and various degrees of intramyocardial block, and therefore a new mechanism of TdP tachycardia in some patients with spontaneous high-degree AVB

Endomyocardial biopsy in patients with acute myocarditis, idiopathic dilated cardiomyopathy, and arrhythmogenic right ventricular dysplasia

Endomyocardial biopsy (EMB) is useful for the diagnosis of myocarditis, cardiac sarcoidosis, and non-ischemic cardiomyopathy. In this mini-review, we discuss the diagnostic potential of EMB in cases of acute/chronic-active myocarditis, sarcoidosis, idiopathic dilated cardiomyopathy and arrhythmogenic right ventricular dysplasia. We also summarize the complications caused by endomyocardial biopsy procedures. Importantly, we finally review the emerging molecular biology technologies as well as biological engineering techniques that can help improve the diagnostic accuracy of EMB to diagnose myocarditis and cardiomyopathies, promoting the management of these diseases

Atrial cardiomyopathy: from cell to bedside

Atrial cardiomyopathy refers to structural and electrical remodelling of the atria, which can lead to impaired mechanical function. While historical studies have implicated atrial fibrillation as the leading cause of cardioembolic stroke, atrial cardiomyopathy may be an important, underestimated contributor. To date, the relationship between atrial cardiomyopathy, atrial fibrillation, and cardioembolic stroke remains obscure. This review summarizes the pathogenesis of atrial cardiomyopathy, with a special focus on neurohormonal and inflammatory mechanisms, as well as the role of adipose tissue, especially epicardial fat in atrial remodelling. It reviews the current evidence implicating atrial cardiomyopathy as a cause of embolic stroke, with atrial fibrillation as a lagging marker of an increased thrombogenic atrial substrate. Finally, it discusses the potential of antithrombotic therapy in embolic stroke with undetermined source and appraises the available diagnostic techniques for atrial cardiomyopathy, including imaging techniques such as echocardiography, computed tomography, and magnetic resonance imaging as well as electroanatomic mapping, electrocardiogram, biomarkers, and genetic testing. More prospective studies are needed to define the relationship between atrial cardiomyopathy, atrial fibrillation, and embolic stroke and to establish a prompt diagnosis and specific treatment strategies in these patients with atrial cardiomyopathy for the secondary and even primary prevention of embolic stroke

Risk Factors Promoting Hypertensive Crises: Evidence From a Longitudinal Study

Background Current knowledge about risk factors promoting hypertensive crisis originates from retrospective data. Therefore, potential risk factors of hypertensive crisis were assessed in a prospective longitudinal study. Methods Eighty-nine patients of the medical outpatient unit at the University Hospital of Bern (Bern, Switzerland) with previously diagnosed hypertension participated in this study. At baseline, 33 potential risk factors were assessed. All patients were followed-up for the outcome of hypertensive crisis. Cox regression models were used to detect relationships between risk factors and hypertensive crisis (defined as acute rise of systolic blood pressure (BP) ≥200mmHg and/or diastolic BP ≥120mmHg). Results The mean duration of follow-up was 1.6 ± 0.3 years (range 1.0-2.4 years). Four patients (4.5%) were lost to follow-up. Thirteen patients (15.3%) experienced hypertensive crisis during follow-up. Several potential risk factors were significantly associated with hypertensive crisis: female sex, higher grades of obesity, the presence of a hypertensive or coronary heart disease, the presence of a somatoform disorder, a higher number of antihypertensive drugs, and nonadherence to medication. As measured by the hazard ratio, nonadherence was the most important factor associated with hypertensive crisis (hazard ratio 5.88, 95% confidence interval 1.59-21.77, P < 0.01). Conclusions This study identified several potential risk factors of hypertensive crisis. Results of this study are consistent with the hypothesis that improvement of medical adherence in antihypertensive therapy would help to prevent hypertensive crises. However, larger studies are needed to assess potential confounding, other risk factors and the possibility of interaction between predictor

Cardiovascular disease during the COVID-19 pandemic: Think ahead, protect hearts, reduce mortality

Coronavirus disease 2019 (COVID-19) is rapidly spreading globally. As of October 3, 2020, the number of confirmed cases has been nearly 34 million with more than 1 million fatalities. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is accountable for COVID-19. Newly diagnosed and worsening cardiovascular disease are common complications in COVID-19 patients, including acute cardiac injury, hypertension, arrhythmia, myocardial infarction, heart failure and sudden cardiac arrest. The mechanisms contributing to cardiac disease burden include hypoxemia, inflammatory factor storm, dysfunctional angiotensin converting enzyme 2 (ACE2), and drug-induced cardiac toxicity.Notably, the macrophages expressing ACE2 as direct host cells of SARS-CoV-2 secrete chemokine and inflammatory cytokines, as well as a decrease in cellular immune responses to SARS-CoV-2 infection due to elevated exhaustion levels and dysfunctional diversity of T cells, that may be accountable for the “hyperinflammation and cytokine storm syndrome” and subsequently acute cardiac injury and deterioratingcardiovascular disease in COVID-19 patients. However, no targeted medication or vaccines for COVID-19 are yet available. The management of cardiovascular disease in patients with COVID-19 include general supportive treatment, circulatory support, other symptomatic treatment, psychological assistance as well as online consultation. Further work should be concentrated on better understanding the pathogenesis of COVID-19 and accelerating the development of drugs and vaccines to reduce the cardiac disease burden and promote the management of COVID-19 patients, especially those with a severe disease course and cardiovascular complications