Advances in arrhythmogenic right ventricular cardiomyopathy
10.3760/cma.j.issn.1673-4408.2010.02.016
- VernacularTitle:致心律失常性右室心肌病研究进展
- Author:
Ping LIN
;
Qijian YI
- Publication Type:Journal Article
- Keywords:
Arrhythmogenic right ventricular cardiomyopathy;
Arrhy thmias;
Sudden death
- From:
International Journal of Pediatrics
2010;37(2):158-161
- CountryChina
- Language:Chinese
-
Abstract:
Arrhythmogenic right ventricular cardiomyopathy(ARVC) is a cardiac muscle disease characterized by peculiar right ventricular involvement that precipitates ventricular arrhythmias and sudden death.ARVC is a major cause of sudden death in the young and athletes.It is familial origin in 50% to 70% of cases,which follows an autosomal in heritance pattern.The pathology consists of a genetically determined dystrophy of the right ventricular myocardium with fibrofatty replacement.Clinical diagnosis can be achieved by demonstrating functional and structural alterations of the right ventricle,depolarization and repolarization abnormalities,arrhythmias with the left bundle branch block and fibro-fatty replacement through endomyocardial biopsy.Clinical research has indicated that successful treatment of ARVC is based on lifestyle modifications,prophylactic therapy(antiarrhythmic drugs,catheter ablation and implantable cardioverter defibrillator)to prevent sudden death.