Arrhythmogenic Right Ventricular Dysplasia

详细信息    查看全文

• 作者：Palaniappan Muthappan ; Hugh Calkins
• 刊名：Progress in Cardiovascular Diseases
• 出版年：2008
• 出版时间：July-August 2008
• 年：2008
• 卷：51
• 期：1
• 页码：31-43
• 全文大小：693 K

文摘

Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV). ARVD results from progressive replacement of right ventricular myocardium with fatty and fibrous tissue. The precise prevalence of ARVD in the United States has been estimated to be 1 in 5000 of the general population. Recent evidence has made it clear that ARVD is a disease of desmosomal dysfunction. The main management consideration concerns whether to implant an ICD. Catheter ablation of VT is a largely a paliative procedure that should not be considered as an appropriate strategy to eliminate VT or reduce sudden death risk. It is likely that the recent advances in the understanding of the pathophysiologic basis of this condition will result in more targeted treatment approaches in the future.