Abstract
Purpose of review: Arrhythmogenic right-ventricular dysplasia is a rare inherited disease characterized by right-ventricular dysfunction and ventricular arrhythmias. The purpose of this article is to review recent developments concerning the diagnosis, genetics, and management of patients with this disease. Recent findings: In the past few years important new information has emerged regarding the role of magnetic resonance imaging in the diagnosis of arrhythmogenic right-ventricular dysplasia. Although magnetic resonance imaging is a very sensitive tool, it is also the most common reason for over diagnosis of this condition. There have also been important new breakthroughs in the genetic basis of arrhythmogenic right-ventricular dysplasia; it now appears that most forms result from mutations in genes encoding desmosomal junction proteins. This may explain why arrhythmogenic right-ventricular dysplasia preferentially impacts the thin right ventricle. Other studies have demonstrated the important role of implantable cardioverter defibrillator therapy in the management of patients with arrhythmogenic right-ventricular dysplasia. In the USA, most patients who meet the Task Force criteria for the disease undergo placement of an implantable cardioverter defibrillator for prevention of sudden cardiac death. Summary: Arrhythmogenic right-ventricular dysplasia is a rare disease. Recent new findings concerning the diagnosis and management of these patients should have direct implications regarding the evaluation and management of patients with this rare, but potentially life-threatening, disorder.
Original language | English (US) |
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Pages (from-to) | 55-63 |
Number of pages | 9 |
Journal | Current opinion in cardiology |
Volume | 21 |
Issue number | 1 |
DOIs | |
State | Published - Jan 2006 |
Keywords
- Arrhythmogenic right-ventricular dysplasia
- Implantable cardioverter defibrillator
- Right-ventricular cardiomyopathy
- Sudden cardiac death
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine