TY - JOUR
T1 - Arrhythmogenic Right Ventricular Dysplasia
AU - Muthappan, Palaniappan
AU - Calkins, Hugh
PY - 2008/7/1
Y1 - 2008/7/1
N2 - 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.
AB - 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.
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U2 - 10.1016/j.pcad.2008.01.002
DO - 10.1016/j.pcad.2008.01.002
M3 - Article
C2 - 18634916
AN - SCOPUS:46849084325
SN - 0033-0620
VL - 51
SP - 31
EP - 43
JO - Progress in Cardiovascular Diseases
JF - Progress in Cardiovascular Diseases
IS - 1
ER -