Arrhythmogenic Right Ventricular Dysplasia (Arrhythmogenic Right Ventricular Cardiomyopathy)

A congenital cardiomyopathy that is characterized by infiltration of adipose and fibrous tissue into the RIGHT VENTRICLE wall and loss of myocardial cells. Primary injuries usually are at the free wall of right ventricular and right atria resulting in ventricular and supraventricular arrhythmias.

Also Known As:

Networked: 673 relevant articles (21 outcomes, 55 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Ventricular Tachycardia
2.	Arrhythmogenic Right Ventricular Dysplasia (Arrhythmogenic Right Ventricular Cardiomyopathy)
3.	Myocardial Infarction
4.	Heart Failure
5.	Brugada Syndrome

Experts

1.	Calkins, Hugh: 53 articles (01/2022 - 05/2004)
2.	Tandri, Harikrishna: 35 articles (01/2022 - 05/2004)
3.	Tichnell, Crystal: 33 articles (01/2022 - 05/2004)
4.	Murray, Brittney: 26 articles (01/2022 - 09/2011)
5.	Thiene, Gaetano: 26 articles (01/2021 - 11/2002)
6.	Basso, Cristina: 25 articles (01/2021 - 11/2002)
7.	James, Cynthia A: 24 articles (01/2022 - 09/2011)
8.	Judge, Daniel P: 22 articles (01/2021 - 03/2006)
9.	McKenna, William J: 22 articles (01/2021 - 03/2004)
10.	Bauce, Barbara: 19 articles (01/2021 - 11/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Arrhythmogenic Right Ventricular Dysplasia:

1.	Anti-Arrhythmia AgentsIBA 08/11/2009 - "This study sought to examine the efficacy of empiric antiarrhythmic drugs in a rigorously characterized cohort of arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. " 08/11/2009 - "Efficacy of antiarrhythmic drugs in arrhythmogenic right ventricular cardiomyopathy: a report from the North American ARVC Registry." 01/01/2021 - "Anti-arrhythmic drugs in arrhythmogenic right ventricular cardiomyopathy: The importance of optimal beta-blocker dose titration." 07/10/2011 - "A 26-year-old Caucasian woman who presented to our hospital with heart palpitations was diagnosed with arrhythmogenic right ventricular dysplasia, and, after three years of follow up with anti-arrhythmic drugs, she wanted to conceive. " 04/19/2023 - "Antiarrhythmic Drug Therapy in Arrhythmogenic Right Ventricular Cardiomyopathy."
2.	CatecholaminesIBA 04/01/1997 - "A differential diagnosis of an inherited long QT interval syndrome, catecholamine-induced arrhythmias, and arrhythmogenic right ventricular dysplasia have been suggested. " 02/01/2013 - "High prevalence of catecholamine-facilitated focal ventricular tachycardia in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy." 02/01/2013 - "Various arrhythmic diseases (such as congenital long or short QT syndrome, Brugada syndrome, catecholamine-sensitive polymorphic ventricular tachycardia, arrhythmogenic right ventricular cardiomyopathy, early repolarization syndrome etc.) are categorized as hereditary arrhythmic diseases. " 02/01/2013 - "These findings highlight the importance of catecholamine challenge and PVC mapping, which can in turn facilitate ablation of the VT in arrhythmogenic right ventricular dysplasia/cardiomyopathy." 02/01/2001 - "In recent in vivo studies using positron emission tomography (PET) our group demonstrated that the myocardial beta adrenoceptor (betaAR) density is reduced in arrhythmogenic right ventricular cardiomyopathy (ARVC) and idiopathic right ventricular outflow tract tachycardia (RVO-VT) associated with an increased presynaptic catecholamine washout. "
3.	Adrenergic Agents (Adrenergic Drugs)IBA 01/01/2022 - "Furthermore, the gene set enrichment analysis highlights the activation of MAPK and 4 cardiomyocyte/cardiomyopathy-related signaling pathways (including adrenergic signaling in cardiomyocytes and arrhythmogenic right ventricular cardiomyopathy) following repeated electronic cigarette use. " 12/01/1995 - "In patients suffering from various arrhythmias such as idiopathic ventricular arrhythmias, arrhythmogenic right ventricular cardiomyopathy or a long-QT syndrome, MIBG imaging has evidenced regional abnormalities of adrenergic nerve function and has provided new insights into the pathophysiological mechanisms of such disorders. " 11/01/1989 - "The authors studied this adrenergic factor prospectively by exercise stress testing and intravenous isoprenaline in 107 patients referred for evaluation of arrhythmias or symptoms thought to be due to arrhythmias: 30 patients had morphologically normal hearts (15 ventricular extrasystoles, 15 bursts of ventricular tachycardia); 55 patients had dilated cardiomyopathy and 22 had probable or proven arrhythmogenic right ventricular dysplasia. " 07/30/2023 - "The pathway enrichment analysis using predicted miR-129-1-5p mRNA targets demonstrated enrichment in adrenergic signaling in cardiomyocytes, arrhythmogenic right ventricular cardiomyopathy, and oxytocin signaling pathways. " 04/04/2000 - "The frequent provocation of ventricular tachycardia by stress or catecholamines and the efficacy of antiarrhythmic drugs with antiadrenergic properties suggest an involvement of the cardiac adrenergic system in arrhythmogenesis in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). "
4.	Flecainide (Tambocor)FDA LinkGeneric 01/01/2022 - "Safety and efficacy of flecainide associated with beta-blockers in arrhythmogenic right ventricular cardiomyopathy." 06/01/1998 - "This is the first reported case of a young woman with a moderate to severe form of arrhythmogenic right ventricular dysplasia under flecainide treatment who, with her physician's consent, decided to have a baby. " 06/01/1998 - "Arrhythmogenic right ventricular dysplasia: pregnancy under flecainide treatment." 05/01/1990 - "Flecainide produced favorable responses in patients of AVRT, nsVT and sVT with arrhythmogenic right ventricular dysplasia (ARVD). " 01/01/2017 - "Use of flecainide in combination antiarrhythmic therapy in patients with arrhythmogenic right ventricular cardiomyopathy."
5.	Angiotensin-Converting Enzyme Inhibitors (ACE Inhibitors)IBA 12/01/2005 - "We also recommend treatment of patients with ARVD/C with beta blockers and angiotensin-converting enzyme inhibitors, and that all patients with ARVD/C be screened for a mutation in the gene for plakophilin-2, because this is present in more than one third of patients with ARVD/C and may be helpful in the management of first-degree relatives." 01/01/2021 - "Angiotensin-Converting Enzyme Inhibitors/Angiotensin Receptor Blockers: Anti-arrhythmic Drug for Arrhythmogenic Right Ventricular Cardiomyopathy."
6.	Carvedilol (Coreg)FDA LinkGeneric 01/01/2004 - "Carvedilol therapy improved left ventricular function in a patient with arrhythmogenic right ventricular cardiomyopathy." 09/01/2015 - "Genes encoding proteins in the tight junction, malaria, and viral myocarditis pathways were upregulated in the LV by carvedilol, while genes encoding proteins in the glycosaminoglycan biosynthesis and arrhythmogenic right ventricular cardiomyopathy pathways were downregulated by carvedilol. "
7.	Fluconazole (Zonal)FDA LinkGeneric 01/01/2021 - "Currently, the VCG still is superior to the ECG in specific situations, such as in the evaluation of myocardial infarctions when associated with intraventricular conduction disturbances, in the identification and location of accessory pathways in ventricular preexcitation, in the differential diagnosis of patterns varying from normal of electrical axis deviation, in the evaluation of particular aspects of Brugada syndrome, Brugada phenocopies, concealed form of arrhythmogenic right ventricular cardiomyopathy and zonal or fascicular blocks of the right bundle branch on right ventricular free wall.VCG allows us to analyze the presence of left septal fascicular block more accurately than ECG and in the diagnosis of the interatrial blocks and severity of some chambers enlargements. "
8.	Peptidyl-Dipeptidase A (Angiotensin Converting Enzyme)IBA 12/01/2008 - "Angiotensin-converting enzyme gene polymorphism in arrhythmogenic right ventricular dysplasia: is DD genotype helpful in predicting syncope risk?"
9.	Glycogen Synthase (Synthase I)IBA 10/01/2019 - "Severe Cardiac Dysfunction and Death Caused by Arrhythmogenic Right Ventricular Cardiomyopathy Type 5 Are Improved by Inhibition of Glycogen Synthase Kinase-3β."
10.	Proteins (Proteins, Gene)FDA Link 01/01/2023 - "The patient underwent a genetic study which showed a mutation in the gene that encodes the desmocolin-2 protein (DSC-2), which is involved in the pathogenesis of arrhythmogenic right ventricular cardiomyopathy (ARVC). " 01/01/2022 - "A previous study found that transmembrane protein 43 (TMEM43) was highly associated with arrhythmogenic right ventricular dysplasia/cardiomyopathy. " 01/01/2013 - "The aim of this study was to characterize the expression profile of desmosomal proteins in explanted ARVD/C heart samples, in order to identify common features of the disease. " 04/04/2006 - "A recent study reported mutations in PKP2, encoding the desmosomal protein plakophilin-2, associated with ARVD/C. " 09/01/2023 - "Transmembrane protein 43 (TMEM43), a member of the transmembrane protein subfamily, was found to be associated with arrhythmogenic right ventricular cardiomyopathy. "

Therapies and Procedures

1.	Catheter Ablation 06/01/2012 - "The overall objective of the present study was to assess the efficacy of radiofrequency catheter ablation (RFA) of VT in ARVD/C, with particular focus on newer ablation strategies, including epicardial catheter ablation. " 10/26/2021 - "Efficacy of Catheter Ablation for Atrial Arrhythmias in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy-A Multicenter Study." 02/01/2013 - "The study population included 16 consecutive arrhythmogenic right ventricular dysplasia/cardiomyopathy patients with recurrent VT who underwent catheter ablation. " 06/01/2003 - "The aim of this study was to characterize endocardial right ventricular activation by electroanatomic mapping as a guide for catheter ablation in patients with arrhythmogenic right ventricular dysplasia. " 01/01/2020 - "Catheter ablation of electrical storm in patients with arrhythmogenic right ventricular cardiomyopathy."
2.	Therapeutics 01/01/2017 - "Endocardial and epicardial electroanatomical mapping and ablation is a safe and effective therapy in the treatment of right ventricle arrhythmias occurring in the setting of arrhythmogenic right ventricular cardiomyopathy (ARVD). " 05/01/2007 - "A novel treatment strategy for therapy refractory ventricular arrhythmias in the setting of arrhythmogenic right ventricular dysplasia." 01/01/2019 - "Evidence for AAD therapy for arrhythmogenic right ventricular dysplasia (ARVD) is limited by its low prevalence and lack of studies. " 01/01/2023 - "Herein, we present a successful implantation of the HeartMate 3 for isolated right ventricular support as destination therapy in a patient suffering from late-onset arrhythmogenic right ventricular cardiomyopathy." 01/01/2022 - "Reply to the Letter to the Editor entitled "The importance of anti-fibrotic drugs as first-line therapy in patients with arrhythmogenic right ventricular dysplasia"."
3.	Implantable Defibrillators (Implantable Cardioverter-Defibrillator) 01/01/2019 - "Despite expanding indication of the subcutaneous implantable cardioverter defibrillator (S-ICD) in clinical practice, limited data exists on safety and efficacy of S-ICD in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. " 04/01/2017 - "Patients underwent a complete history investigation, physical examination, laboratory studies, echocardiography, electrocardiography, treadmill test, and Holter monitoring before endomyocardial biopsy; two patients with arrhythmogenic right ventricular dysplasia had implantable cardioverter defibrillator implantation and further appropriate successful device shocks. " 05/01/2007 - "The aim of the present study was to investigate outcomes of implantable cardioverter-defibrillator (ICD) treatment in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). " 12/01/2023 - "Implantable cardioverter-defibrillator shocks during long-term follow-up in arrhythmogenic right ventricular cardiomyopathy." 01/01/2023 - "Additional left ventricular septal lead facilitates R-wave sensing of implantable cardioverter-defibrillator in arrhythmogenic right ventricular cardiomyopathy: a case report."
4.	Radiofrequency Ablation 01/01/2017 - "Radiofrequency Ablation in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)." 01/01/2003 - "Non-pharmacological treatment by radio-frequency ablation and implantable defibrillators will play an increasing role in the treatment of patients with ARVD/C, especially in case of drug ineffectivity. " 01/01/2018 - "Studies assessing radiofrequency ablation (RFA) of ventricular tachycardia (VT) in arrhythmogenic right ventricular cardiomyopathy (ARVC) report VT recurrences, but have not evaluated the impact of RFA on relevant clinical events during follow-up. " 01/01/2016 - "Right ventricular thrombus formation in a patient with arrhythmogenic right ventricular dysplasia following radiofrequency ablation." 04/01/2007 - "Radiofrequency ablation of the ventricular tachycardia with arrhythmogenic right ventricular cardiomyopathy using non-contact mapping."
5.	Cardiac Resynchronization Therapy 11/01/2012 - "Rare case of left-dominant arrhythmogenic right ventricular cardiomyopathy with dramatic reverse remodeling after cardiac resynchronization as an adjunct to pharmacological therapy." 08/30/2007 - "Finally, clinical examples of deformation imaging in hypertrophic cardiomyopathy (HCM), cardiac resynchronization therapy (CRT) and arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) are presented." 10/01/2017 - "We go on to demonstrate how these modelling approaches in combination with cardiac imaging have improved understanding of RV pump function in pulmonary arterial hypertension, arrhythmogenic right ventricular cardiomyopathy, dyssynchronous heart failure and cardiac resynchronization therapy, hypoplastic left heart syndrome, and repaired tetralogy of Fallot. "