Recent advances in molecular genetics of arrhythmogenic right ventricular cardiomyopathy (ARVD) are reviewed. In particular, the finding of mutations in the gene coding for cardiac ryanodine receptor (hRYR2), both in patients affected with ARVD2 and in patients affected with catecholaminergic ventricular arrhythmias or with familial ventricular tachyarrhythmia, is discussed. Novel data support ...

A 27-year-old man who was diagnosed with arrhythmogenic right ventricular dysplasia (ARVD) underwent the total right ventricular (RV) exclusion procedure: the RV free wall was resected and an extracardiac total cavopulmonary connection (TCPC) was created using a 24-mm-diameter polytetrafluoroethylene (PTFE) graft. After an uneventful period of 7 years, he began to develop protein-losing enterop...

Arrhythmogenic right ventricular dysplasia is an inherited, progressive condition. Characterised by fatty infiltration of the right ventricle, it frequently results in life threatening cardiac arrhythmias, and is one of the important causes of sudden cardiac death in the young. There are characteristic electrocardiographic and echocardiographic features that all physicians need to be aware of i...

Reprint requests to: Dr. Wen-Sheng Tzeng Department of Diagnostic Radiology, Chi-Mei Medical Center, Liou-Ying Campus. No. 201, Taikang Village, Liou-Ying, Tainan 736, Taiwan, R.O.C. Arrhythmogenic right ventricular dysplasia (A RV D) i s a non i schem ic c ard iomyopathy involving primarily the right ventricle. Its characteristics include hypokinetic areas involving the free wall of the right ...