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Author(s): Rajiv Sankaranarayanan , Eleanor J Fleming , Clifford J Garratt Added: 3 years ago
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disorder worldwide with a prevalence of 1 in 500 in the general population.1 It can be defined as a condition that is typified by unexplained left ventricular hypertrophy (LVH) in the absence of other cardiac or non-cardiac conditions that could produce hypertrophy of similar proportions.2 The condition was the first… View more
Author(s): Amar Trivedi , Bradley P Knight Added: 3 years ago
Hypertrophic cardiomyopathy (HCM), a genetic sarcomeric disorder associated with myocyte disarray and scar deposition, is intimately linked to sudden cardiac death (SCD) due to malignant ventricular arrhythmias. In the first modern published description of the disease in 1958, Dr Donald Teare describes the case of a 14-year-old male who collapsed while being chased around his school’s playground… View more
Author(s): Saagar Mahida Added: 3 years ago
Atrial fibrillation (AF) is a highly prevalent arrhythmia that represents an important burden on healthcare systems. The presence of AF is associated with an increased risk of conditions such stroke, heart failure and dementia. Further, AF is associated with increased mortality. Over the past half century, significant advances have been made in understanding the pathobiology of AF. Important… View more
Author(s): Deniz Akdis , Corinna Brunckhorst , Firat Duru , et al Added: 3 years ago
Arrhythmogenic cardiomyopathy (ACM) is usually referred to as arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).1A first historical description was made in 1736, whereas its first modern description dates back to 1982.2 Initially, ACM was thought to be an embryological malformation.3 Yet in recent years it became evident that the pathophysiology of an ongoing genetically… View more
Author(s): Moinuddin Choudhury , Mark R Boyett , Gwilym M Morris Added: 3 years ago
The sinoatrial or sinus node (SAN) is the heart’s natural pacemaker. Located in the superior right atrium, it automatically produces cyclical electrical activity to initiate each heartbeat in normal sinus rhythm. SAN dysfunction (SND) in humans, also known as ‘sick sinus syndrome’, can manifest as pathological bradycardia and asystolic pauses. As a result, SND can lead to symptoms of reduced… View more
Author(s): Brenton S Bauer , Anthony C Li , Jason S Bradfield Added: 3 years ago
Ventricular arrhythmias (VA) are commonly associated with structural heart disease and have substantial impact on patient outcomes and health system costs. Within the realm of cardiomyopathy (CM), there has been substantial progress with respect to ischaemic CM (ICM) in the understanding of infarct related scar biology and scar-mediated ventricular tachycardia (VT). This has led to interventions… View more
Author(s): Philippe Charron , Eloisa Arbustini , Gisèle Bonne Added: 3 years ago
Most mutations of the LMNA gene affect the heart, causing a dilated cardiomyopathy, ususally with conduction defect and ventricular arrhythmia, with or without skeletal muscle involvement. Although a relatively rare disease, cardiologists should be aware of laminopathies (diseases caused by LMNA gene mutations) because of the particularly aggressive course compared with most other… View more
Author(s): Dario DiFrancesco Added: 3 years ago
HCN4 (hyperpolarisation-activated, cyclic nucleotide gated 4) channels, the pore-forming α–subunits of ‘funny’ channels originally described in pacemaker cells of the sinoatrial node (SAN),1 are responsible for the early phase of diastolic depolarisation in these cells and are key determinants of pacemaker generation and control of heart rate.2–5 HCN4 channels are selectively expressed in the SAN… View more
Author(s): Giulio Conte , Maria Luce Caputo , François Regoli , et al Added: 3 years ago
General understanding of early repolarisation (ER) has dramatically changed in the last decade. For several years, ER has been considered a benign electrocardiographic (ECG) finding with high prevalence in the general population. Recently different studies have challenged this view and showed a significant association with life-threatening arrhythmias.1–5 In 2008 Haïssaguerre et al. first… View more
Author(s): Cynthia A James Added: 3 years ago
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare heritable cardiomyopathy characterised by fibro-fatty replacement of the myocardium, which predisposes patients to frequent lifethreatening ventricular arrhythmias and slowly progressive ventricular dysfunction.1,2 Structural involvement of the right ventricle (RV) generally predominates,3,4 although left dominant forms… View more