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Arrhythmias
Published in Clive Handler, Gerry Coghlan, Nick Brown, Management of Cardiac Problems in Primary Care, 2018
Clive Handler, Gerry Coghlan, Nick Brown
Arrhythmogenic right ventricular dysplasia (ARVD) associated with ventricular tachycardia or ventricular fibrillation, extensive disease on echocardiography, or a family history of sudden death or unexplained syncope.
Ablation of ventricular arrhythmias
Published in Ever D. Grech, Practical Interventional Cardiology, 2017
During ischaemic injury, the wavefront of necrosis traverses from endo- to epicardium. Hence, mapping and ablation of ischaemic VT targets endocardial scar and rarely necessitates an epicardial approach.14 Epicardial mapping and ablation is indicated in patients with ARVD. In these patients, scar formation initiates from the epicardium and advances towards the endocardium. Epicardial access is obtained first in patients with a high likelihood for an epicardial substrate, since endocardial mapping will require heparinisation to prevent intra-cardiac thrombus formation. In DCM, an endocardial approach as the initial step may be preferred. Only if the clinical VT demonstrates typical morphology of an epicardial origin, should a combined epi- and endocardial approach serve as the initial step.15 Endocardial bipolar voltage signals provide accurate detail of tissue composition within the immediate vicinity (near field) of the distal electrode pair of the ablation catheter. Extending the range of view, endocardial unipolar voltage recorded between the distal tip of the ablation catheter and Wilson's central terminal may identify epicardial scar in patients with ARVD or non-ICM supporting the need for additional epicardial mapping and ablation.16,17
The cardiomyopathies
Published in Andrew R. Houghton, MAKING SENSE of Echocardiography, 2013
Arrhythmogenic RV cardiomyopathy or dysplasia (ARVC or ARVD) is a rare hereditary cardiomyopathy, primarily affecting the RV, in which there is loss of myocytes and replacement with fatty/fibrous tissue. Patients may present with ventricular arrhythmias; sudden cardiac death may occur.
Overview and surgical aspects of Uhls anomaly
Published in Expert Opinion on Orphan Drugs, 2020
Kotturathu Mammen Cherian, Lydia Jeris W, Mohamed Thayub
Uhls anomaly is very rare and very few cases have been reported. It is characterized by partial or total loss of myocardium of right ventricle leading on to a thin-walled dilated right ventricle, hence also called Parchment paper ventricle. Uhls anomaly usually occurs in isolation but can be associated with other congenital malformations. The age at presentation is usually infancy but can present at any age and is picked up many times at autopsy only. The presentation can be varied ranging from asymptomatic status to right heart failure, arrhythmia, pleural effusion, cardiac tamponade, and so on. Cyanosis is usually noted as there is shunting of blood from right to left. A closely related anomaly is “Arrhythmogenic right ventricular dysplasia” (ARVD) wherein there is fibrofatty replacement of right ventricular myocardium which is not seen in case of Uhls anomaly. ECHO, MRI, and histopathological examination aid in the diagnosis of Uhls anomaly.
Parameters affecting outcome of paediatric cardiomyopathies in the intensive care unit: experience of an Egyptian tertiary centre over 7 years
Published in Libyan Journal of Medicine, 2020
Alaa A. Sobeih, Sonia A. El-Saiedi, Noha S. Abdel Khalek, Shereen A. Attia, Baher M. Hanna
A total of 92 patients were included in the study, representing 8.6% of the total admissions (n = 1071) to the PCICU. Males represented 55.4% of the patients and females were 44.6% (n = 51 and 41, respectively). The mean age at admission was 20.5 months (range 6–144 months; median 72 months), and the mean weight was 20.5 kg (range 4.5–55 kg). The types of cardiomyopathy included DCM (n = 80, 87%), HCM (n = 6, 6.5%), RCM (n = 4, 4.3%) and mixed DCM-RCM (n = 2, 2.2%). There were no cases of LVNC or arrhythmogenic right ventricular dysplasia (ARVD) encountered during the period of study. Twelve per cent of the patients were readmitted once, and 5.4% were readmitted more than once. The survival rate was 75% among our patients (n = 69). The most common cause of mortality was intractable heart failure (in about 63%); followed by malignant arrhythmias in 21%; and infections in 10%. A comparison of survivors with non-survivors showed no statistically significant correlation with the type of cardiomyopathy, gender, age, weight, height, or body/surface area (BSA).
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].