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Arrhythmogenic right ventricular dysplasia/cardiomyopathy.
MedLine Citation:
PMID:  16283974     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV). The most important aspect in the treatment of ARVD/C is establishing a correct diagnosis based on the International Task Force criteria. In our experience, cardiologists are not aware of these diagnostic criteria for ARVD/C and place too much importance on the results of magnetic resonance imaging of the RV. Patients with ARVD/C generally all have an abnormal 12-lead electrocardiogram, abnormal echocardiogram, and ventricular arrhythmias with a left bundle branch block morphology. If noninvasive testing suggests ARVD, invasive testing with an RV angiogram, RV biopsy, and electrophysiology study are recommended. We encourage patients to participate in the National Institutes of Health-sponsored multicenter clinical trial of ARVD/C (http://www.ARVD.comor Once a diagnosis of ARVD/C is established, the main treatment decision involves whether to implant an implantable cardioverter-defibrillator (ICD). ICDs are recommended for patients who have experienced syncope, sudden death, or a sustained ventricular arrhythmia, and also for patients with overt evidence of ARVD, particularly if the electrophysiology study is abnormal or there is a family history of sudden death. 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.
Kalpana R Prakasa; Hugh Calkins
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Current treatment options in cardiovascular medicine     Volume:  7     ISSN:  1534-3189     ISO Abbreviation:  Curr Treat Options Cardiovasc Med     Publication Date:  2005 Dec 
Date Detail:
Created Date:  2005-11-14     Completed Date:  2007-07-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9815942     Medline TA:  Curr Treat Options Cardiovasc Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  467-75     Citation Subset:  -    
Carnegie 592, Johns Hopkins Hospital, 600, N. Wolfe Street, Baltimore, MD 21287, USA.
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