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Recognition of common arrhythmias
Published in John Edward Boland, David W. M. Muller, Interventional Cardiology and Cardiac Catheterisation, 2019
Nicholas P. Kerr, Rajesh N. Subbiah
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a potentially lethal structural cardiomyopathy that presents with MMVT. The diagnosis can be difficult to make because patients may present with VT or sudden cardiac arrest prior to overt structural cardiomyopathy being evident on imaging studies. VT in ARVC has a LBBB pattern in lead V1, indicating an origin in the right ventricle. There are usually multiple VT morphologies, often with different frontal plane axes. The sinus rhythm 12-lead ECG frequently has important characteristic features, including an incomplete RBBB pattern and epsilon wave (terminal notch in the QRS representing delayed conduction into the right ventricular outflow tract (RVOT) as well as T-wave inversions in the right praecordial leads. Cardiac magnetic resonance imaging to evaluate for ARVC should be performed in any patient without apparent structural heart disease with a VT morphology suggesting an origin in the RV. Management of patients with ARVC and VT requires an ICD, with antiarrhythmic mediations, and occasionally epicardial VT ablation used to control recurrent VT episodes.
Cardiology
Published in Ashley Bond, MRCP Part 2 Examination, 2017
Features: SCD and VTdilated RVT wave inversion V1–V3epsilon wave seen at end of QRS.
Non-ischemic Cardiomyopathy and Ventricular Arrhythmias
Published in Andrea Natale, Oussama M. Wazni, Kalyanam Shivkumar, Francis E. Marchlinski, Handbook of Cardiac Electrophysiology, 2020
Matthew C. Hyman, Gregory E. Supple
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is unique from most other forms of non-ischemic cardiomyopathy in that the bulk of the arrhythmogenic substrate is found in the right ventricle. ARVC is characterized by fibrofatty replacement of the myocardial wall typically in the epicardial RVOT or the lateral tricuspid annulus.42–44 The fibrofatty replacement leads to delayed conduction and repolarization abnormalities that are visible on the surface electrocardiogram and have become incorporated in the diagnostic criteria for this disease.45 T-wave inversions are often found over the right precordial leads (V1 and V2), but this inversion can extend throughout the precordium. Late fractionation may be present as well in the right precordium and is termed an epsilon wave (Figure 27.5). Ventricular arrhythmias arising from the ARVC population are predominantly found in the free wall of the RVOT where they manifest with a left bundle morphology with late transition (V5 or V6). There will be an inferior axis that frequently shows notching in the inferior leads. Occasionally, the lateral tricuspid annulus will have aneurysmal formation and can be an additional site of arrhythmias. Lateral tricuspid annulus arrhythmias will be left bundle arrhythmias with a late transition (V5 or V6), but the inferior leads will frequently be biphasic in lead II and negative in lead III. One notable aspect of the ARVC population is that while their heart failure course may be progressive, the arrhythmic substrate has been shown to be relatively static over time.44 This suggests that with a successful initial ablation, ARVC patients can be ventricular arrhythmia free. Achieving successful ablation with endocardial ablation alone can be difficult due to the highly mobile nature of the RV free wall and limited energy delivery to the fibrofatty and epicardial substrate. In a large cohort, 1 year VT free survival is approximately 50% with endocardial ablation alone.46 When the epicardial substrate is targeted in conjunction with the endocardium, VT free survival is reported to between 64% and 77% at 1 year.43,46
Right ventricular dysplasia: management and treatment in light of current evidence
Published in Journal of Community Hospital Internal Medicine Perspectives, 2018
Amr Idris, Syed Raza Shah, Ki Park
Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as ‘Arrhythmogenic right ventricular dysplasia’, is a rare cardiovascular disease, often familial, that predisposes to ventricular arrhythmias (VA) potentially leading to sudden cardiac death (SCD) in young patients [1–4]. ARVC is more prevalent in young people and athletes [5–7]. ARVC was first described in the literature back in the eighteenth century by the Pope’s physician that reported four generations in one family having progressive congestive heart failure with unexplained SCD [8]. This was followed by a case series by Marcus et al., which reported 24 patients with ventricular tachycardia (VT) having left bundle branch block [9]. Additionally, the electrocardiographic epsilon (σ) wave was first described by Fontaine which helped enhance recognition of potential electrocardiographic features of ARVC [10]. Epsilon wave can be seen in the ECG as a small positive deflection (blip) buried in the end of the QRS complex helping identify and diagnose patients with ARVC [10].
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
This patient was initially diagnosed as cardiac involvement of SSc. However, high dose of oral prednisone and intravenous cyclophosphamide did not work. Considering the family history of heart failure, primary cardiomyopathy could not be completely excluded. Further dynamic electrocardiogram monitor of the patient recorded 1526 extrasystoles originated from right ventricle within 24 h, as well as Epsilon wave in the right precordial leads V1 and V2 (Figure 1, Panel D). According to the international Task Force criteria for the clinical diagnosis of ARVC [9], this patient met two major criteria, including cardiac dysfunction and structural alterations confirmed by echo and cardiac magnetic resonance (Figure 1(B,C)), conduction abnormality (Epsilon wave in the right precordial leads V1 and V2, as shown in Figure 1(D)), and a minor criterion, >500 ventricular extrasystoles per 24 h (Holter). Thus, this patient fully fulfilled the definite diagnosis criteria for ARVC.