Key result
Establishing an Asian registry for arrhythmogenic right ventricular dysplasia/cardiomyopathy is urgently needed to investigate its unique genetic and clinical features.
Highlights the need for an Asian registry for ARVD/C to better understand its unique genetic and clinical features in this population.
Arrhythmogenic right venticular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy with a very low penetrance affecting the right ventricle (RV) and presenting palpitation and syncope due to ventricular tachycardia (VT) originating from RV. The VT can degenerate into ventricular fibrillation and sudden cardiac death. The genetic background of ARVD/C has recently been found to be heterogeneous, mainly resulting from cell adhesion abnormalities due to mutations in five different genes encoding members of the desmosome complex. In Asian countries, however, the genetic aspect of the disease has not been fully studied, although the clinical features of Asian ARVD/C patients are different from those in Western countries in the penetrance of phenotypes, relation to Brugada syndrome and link to RV outflow tract ventricular tachycardia. It is of urgent need to have a registry of Asian ARVD/C patients and to conduct a more detailed genetic survey on the candidate genes, including desomosomal ones.
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Horie et al. (2008) conducted an editorial in Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Establishing an Asian registry for arrhythmogenic right ventricular dysplasia/cardiomyopathy is urgently needed to investigate its unique genetic and clinical features.
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