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Arrhythmogenic Right Ventricular Cardiomyopathy
Published in Andrea Natale, Oussama M. Wazni, Kalyanam Shivkumar, Francis E. Marchlinski, Handbook of Cardiac Electrophysiology, 2020
Daniele Muser, Pasquale Santangeli
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined cardiomyopathy clinically characterized by life-threatening ventricular arrhythmias (VAs) and/or heart failure (HF) predominantly involving the right ventricle (RV).1 From its initial description in 1977, several progress have been made in the comprehension of the disease etiology, pathogenesis, clinical manifestations, risk stratification, and management.2 Currently, ARVC is seen as a polygenic-multifactorial disease in which the interaction between a pool of genetic mutations rather than a single gene with other environmental and individual factors such as age, sex, physical activity, infections, and pro-inflammatory conditions leads to the phenotypic manifestation of the disease.1
The QRS complex
Published in Andrew R Houghton, Making Sense of the ECG, 2019
There is no single diagnostic test for ARVC, and the diagnosis is based upon a combination of major and minor criteria, which include ECG findings, family history, arrhythmias, tissue characteristics on endomyocardial biopsy and imaging criteria (for more details see the Task Force Criteria in Marcus et al. in ‘Further reading’).
Cardiology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is very rare in the first decade of life and tends to be a problem of teenagers and young adults, with a high risk of sudden death. Although predominantly a disease of the right ventricle, the left can also be involved. It is autosomal dominantly inherited but genetically heterogeneous.
Risk stratification for ventricular arrhythmias and sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy: an update
Published in Expert Review of Cardiovascular Therapy, 2019
Julia Cadrin-Tourigny, Laurens P Bosman, Rafik Tadros, Mario Talajic, Lena Rivard, Cynthia A James, Paul Khairy
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined cardiomyopathy characterized by progressive scarring of the myocardium. It primarily manifests as ventricular arrhythmia (VA), which makes it a frequent cause of sudden cardiac death (SCD) in young individuals and athletes [1]. Prevention of SCD is the cornerstone in the management of this disease and since implantable cardioverter-defibrillators (ICDs) are the only reliable preventive treatment, the decision to implant an ICD is often the single most important decision in the management of these patients. While the use of ICDs in secondary prevention for ARVC is widely accepted, identifying a high-risk population for primary prevention ICDs still remains a challenge for clinicians. Anticipated benefits must be carefully weighed against the potential short and long-term risks in this young population.
Disease modeling of cardiac arrhythmias using human induced pluripotent stem cells
Published in Expert Opinion on Biological Therapy, 2019
Wenbin Liang, Lilit Gasparyan, Wael AlQarawi, Darryl R. Davis
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive heart muscle disease characterized by fibrofatty replacement of working myocardium. It primarily affects the right ventricle; however, the left ventricle is also frequently involved and often may predominate [133]. Patients usually present between the second and fourth decade of life with ventricular arrhythmias originating from the right ventricle although sudden cardiac death can be the first presentation [134]. The prevalence of ARVC is estimated to be 1 in 5000, affecting men more than women with a ratio of 3 to 1 [135]. The natural history of ARVC is highly variable with a wide spectrum of clinical presentations that likely reflect the diversity of causes. Four patterns of clinical presentation have been proposed [136,137]: (1) A concealed form with no clear structural abnormalities where the diagnosis is usually made during family screening [138]. (2) An overt electrical form, which is the most typical presentation in young patients [139]. (3) Right ventricular failure in which the myocardial fibrofatty replacement results in right ventricular pump failure. (4) Biventricular failure, which usually develops late in the natural history of the disease, and is characterized by early progressive dilatation of both ventricles [140].
Systemic sclerosis complicated by arrhythmogenic right ventricular cardiomyopathy in a Chinese middle-aged female
Published in Modern Rheumatology Case Reports, 2018
Shi-Kun Ma, Chao-Xia Lu, Wei Wu, Yong-Tai Liu, Xue Zhang
Systemic Sclerosis (SSc) is a systematic autoimmune disease affecting multiple organs. The heart is one of its target organs. SSc-associated cardiac abnormalities included microvascular coronary artery disease leading to resultant myocardial ischaemia, myocardial fibrosis, left ventricular (LV) systolic dysfunction, LV diastolic dysfunction, pericardial disease and conduction abnormalities (including bradyarrhythmias and tachyarrhythmias) [1]. These cardiovascular alterations can be primary (i.e. direct cardiac involvement) or secondary to potential complications of SSc, like pulmonary arterial hypertension, interstitial lung disease or kidney disease [2]. ARVC is an autosomal dominant inherited disease with incomplete penetrance and variable clinical phenotype, characterised by right ventricular cardiomyopathy with progressive replacement of myocardium with fatty and fibrous tissue [3]. It is one of the leading causes of sudden cardiac death in the young people [4].