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: 339 relevant articles (4 outcomes, 22 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Ventricular Tachycardia
2.	Syncope (Fainting)
3.	Cardiomyopathies (Cardiomyopathy)
4.	Brugada Syndrome
5.	Long QT Syndrome

Experts

1.	Basso, Cristina: 18 articles (04/2015 - 11/2002)
2.	Thiene, Gaetano: 17 articles (04/2015 - 11/2002)
3.	Calkins, Hugh: 16 articles (08/2014 - 05/2004)
4.	McKenna, William J: 14 articles (08/2012 - 03/2004)
5.	Bauce, Barbara: 12 articles (04/2015 - 11/2002)
6.	Nava, Andrea: 12 articles (12/2013 - 11/2002)
7.	Rampazzo, Alessandra: 11 articles (01/2015 - 11/2002)
8.	Saffitz, Jeffrey E: 10 articles (11/2014 - 05/2004)
9.	Asimaki, Angeliki: 10 articles (11/2014 - 09/2006)
10.	Wichter, Thomas: 10 articles (02/2011 - 08/2002)

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." 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). "
2.	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. "
3.	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?"
4.	CatecholaminesIBA 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). "
5.	gamma Catenin (Plakoglobin)IBA 12/01/2014 - "A recent study using an anti-plakoglobin antibody and immunofluorescence methods in endomyocardial tissue specimens found that a marked reduction in plakoglobin staining was highly sensitive and specific for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC). " 04/01/2011 - "Recent immunohistochemical studies observed the loss of plakoglobin (PG) from the intercalated disc (ID) as a hallmark of arrhythmogenic right ventricular cardiomyopathy (ARVC), suggesting a final common pathway for this disease. " 03/01/2015 - "Plakoglobin: a diagnostic marker of arrhythmogenic right ventricular cardiomyopathy in forensic pathology?" 12/01/2014 - "Plakoglobin immunolocalization as a diagnostic test for arrhythmogenic right ventricular cardiomyopathy." 09/01/2013 - "On the diagnostic utility of junction plakoglobin in arrhythmogenic right ventricular cardiomyopathy."
6.	Desmoplakins (Desmoplakin)IBA 08/01/2012 - "Electrophysiological abnormalities precede overt structural changes in arrhythmogenic right ventricular cardiomyopathy due to mutations in desmoplakin-A combined murine and human study." 08/01/2005 - "Desmoplakin disease in arrhythmogenic right ventricular cardiomyopathy: early genotype-phenotype studies." 06/01/2015 - "Epsilon wave uncovered by exercise test in a patient with desmoplakin-positive arrhythmogenic right ventricular cardiomyopathy." 08/01/2014 - "Desmoplakin (DSP), an arrhythmogenic right ventricular cardiomyopathy gene, contributed 2.4%, illustrating the utility of multidisease testing. " 08/01/2005 - "To characterize the clinical profile of patients belonging to families affected with autosomal dominant arrhythmogenic right ventricular cardiomyopathy (ARVC) due to mutations of the gene encoding for the cell-to-cell adhesion protein desmoplakin (DSP). "
7.	Uhl anomalyIBA 05/01/1985 - "Two-dimensional echocardiographic, radionuclide angiographic, and contrast ventriculographic studies were typical of the right ventricular abnormalities in both Uhl's anomaly and arrhythmogenic right ventricular dysplasia. " 05/01/1983 - "Echocardiographic, radionuclide ventriculographic, and angiographic studies were typical of the right ventricular abnormalities in Uhl's anomaly; electrocardiographic and electrophysiologic findings were those of arrhythmogenic right ventricular dysplasia. " 11/01/2010 - "In particular, Uhl's anomaly could be misdiagnosed as arrhythmogenic right ventricular cardiomyopathy due to the similarity of clinical and echocardiographic findings." 01/01/2009 - "We report on two boys, aged 18 and 17 years, one with arrhythmogenic right ventricular cardiomyopathy (ARVC) and one with Uhl's anomaly. " 02/01/1993 - "Of these, "Uhl's anomaly" and "arrhythmogenic right ventricular dysplasia" are most often used. "
8.	Polymorphic catecholergic ventricular tachycardiaIBA 01/01/2014 - "Based on the clinical characteristics of our case, presenting some overlapping clinical features common to both the diseases, the discussion will be focused on Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) and Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D). " 04/01/2008 - "Several disease-linked mutations of RyR have been reported in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) or arrhythmogenic right ventricular cardiomyopathy type 2 (ARVC2). " 09/01/2005 - "More recently, a considerable number of disease-linked mutations in the RyR have been reported in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) or arrhythmogenic right ventricular dysplasia type 2. An analysis of the disposition of these mutation sites within well-defined domains of the RyR polypeptide chain has led to the new concept that interdomain interactions among these domains play a critical role in channel regulation, and an altered domain interaction causes channel dysfunction in the failing heart. " 07/01/2005 - "The observation of both arrhythmogenic right ventricular cardiomyopathy type 2 and catecholaminergic polymorphic ventricular tachycardia in the same family suggests that the two entities might correspond to different degrees of phenotypic expression of the same disease. " 06/01/2013 - "We studied the prevalence, clinical phenotypes, and risk of SCD presented by ten rare mutations previously associated with arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, or catecholaminergic polymorphic ventricular tachycardia. "
9.	Paroxysmal ventricular fibrillationIBA 08/01/1996 - "Idiopathic ventricular fibrillation of the Brugada type: an atypical form of arrhythmogenic right ventricular cardiomyopathy?" 05/01/1994 - "Potential role of endomyocardial biopsy in the clinical characterization of patients with idiopathic ventricular fibrillation: arrhythmogenic right ventricular dysplasia--an undervalued cause." 04/01/1994 - "Appropriate ICD therapies during mean follow-up periods of 12 to 36 months occurred in 30% of patients with idiopathic ventricular fibrillation to 50% of patients with arrhythmogenic right ventricular cardiomyopathy and long QT syndrome. " 04/01/1994 - "This review summarizes data regarding ICD therapy in patients with arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, and idiopathic ventricular fibrillation and presents preliminary concepts for identification of patients who will benefit more from ICD therapy than from pharmacologic and other nonpharmacologic approaches. " 03/20/2007 - "In this study, we analyzed 83 Japanese patients including patients with long-QT syndrome, Brugada syndrome, idiopathic ventricular fibrillation, arrhythmogenic right ventricular cardiomyopathy and CPVT. "
10.	SodiumIBA 01/01/2012 - "Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with remodeling of gap junctions and also, although less well-defined, down-regulation of the fast sodium current. " 03/11/2014 - "Previous studies showed features consistent with sodium current (INa) deficit in patients carrying desmosomal mutations, diagnosed with arrhythmogenic cardiomyopathy (or arrhythmogenic right ventricular cardiomyopathy). "

Therapies and Procedures

1.	Implantable Defibrillators (Implantable Cardioverter-Defibrillator) 01/01/2010 - "This study investigated the clinical course of arrhythmogenic right ventricular cardiomyopathy (ARVC) patients and in particular evaluated the contribution of radiofrequency catheter ablation (RFCA) and an implantable cardioverter-defibrillator (ICD) to the treatment of ARVC. " 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). " 01/01/2006 - "Other studies have demonstrated the important role of implantable cardioverter defibrillator therapy in the management of patients with arrhythmogenic right-ventricular dysplasia. " 10/01/2014 - "Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular cardiomyopathy: the course of electronic parameters, clinical features, and complications during long-term follow-up." 07/01/2014 - "Long-term prognosis of patients with arrhythmogenic right ventricular cardiomyopathy and implantable defibrillator."
2.	Defibrillators (Defibrillator) 07/01/2015 - "Inappropriate shocks by subcutaneous defibrillator in a patient with arrhythmogenic right ventricular cardiomyopathy: problem fixed." 01/01/2013 - "We report a case of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) which illustrates the natural progression of disease in the absence of availability of an implanted cardiac defibrillator (ICD). " 11/01/2011 - "Defibrillator was implanted in 4, including 2 for arrhythmogenic right ventricular cardiomyopathy diagnosed later. " 08/01/2010 - "Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease where therapy with an internal cardioverter defibrillator (ICD) is frequently indicated, but the use of these devices is often challenged because of sensing dysfunction with low right ventricular sensing amplitude. " 08/01/2010 - "Left ventricular sensing and pacing for sensing difficulties in internal cardioverter defibrillator therapy for arrhythmogenic right ventricular cardiomyopathy."
3.	Heart Transplantation (Grafting, Heart) 11/01/2014 - "Fontan-like circulation as a criterion for heart transplantation in arrhythmogenic right ventricular dysplasia." 04/01/2009 - "Heart transplantation in arrhythmogenic right ventricular dysplasia: case reports." 12/01/2005 - "Three cases of orthotopic heart transplantation for arrhythmogenic right ventricular cardiomyopathy." 02/01/2005 - "Arrhythmogenic right ventricular dysplasia: catheter ablation, MRI, and heart transplantation." 04/01/2014 - "Anesthesia for heart transplantation in patients with arrhythmogenic right ventricular dysplasia."
4.	Transplants (Transplant) 06/01/2010 - "Comparison between electroanatomic and pathologic findings in a patient with arrhythmogenic right ventricular cardiomyopathy/dysplasia treated with orthotopic cardiac transplant." 11/01/2006 - "Post-transplant arrhythmogenic right ventricular cardiomyopathy diagnosis was made in 6 patients with a pre-transplant diagnosis of IDC or KaShan disease. " 03/01/2007 - "Remodeling has been associated with such diseases as pulmonary hypertension, lung transplant, LV pathology, Chagas' disease, and arrhythmogenic right ventricular cardiomyopathy. " 09/01/2014 - "The primary diseases before transplant were idiopathic dilated cardiomyopathy in 33 patients (66%), hypertrophic cardiomyopathy in seven (14%), restrictive cardiomyopathy in one, arrhythmogenic right ventricular cardiomyopathy in one, and secondary cardiomyopathy in eight (16%). " 06/01/2006 - "A 27-year-old man who was diagnosed with arrhythmogenic right ventricular dysplasia (ARVD) underwent the total right ventricular (RV) exclusion procedure: the RV free wall was resected and an extracardiac total cavopulmonary connection (TCPC) was created using a 24-mm-diameter polytetrafluoroethylene (PTFE) graft. "
5.	Homologous Transplantation (Allograft) 05/01/2011 - "This study suggests the fibrofatty infiltration in cardiac allografts is a clinically different entity from arrhythmogenic right ventricular dysplasia and has an unknown etiology. "