Mapping the Electrical Substrate in Arrhythmogenic Right Ventricular Cardiomyopathy
There Is More Than Meets the Eye
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- arrhythmogenic right ventricular cardiomyopathy
- magnetic resonance imaging
- ventricular tachycardia
Arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) is an inherited biventricular cardiomyopathy with a high prevalence of ventricular arrhythmias. Mutations in the cardiac desmosomes lead to abnormal cell-to-cell mechanoelectric coupling, leading to the characteristic ECG and structural manifestations of the disease.1,2 Advances in cardiac magnetic resonance imaging, such as scar imaging and quantitative analyses, have significantly improved the diagnostic capability1,3; however, the diagnosis is still challenging in the early and concealed phases of the disease where the sudden death risk seems to be at its peak.4 The key to early diagnosis seems to be in better definition of the electric substrate because studies have shown that the electric abnormalities, such as depolarization and repolarization changes, precede structural changes in ARVC. ECG imaging (ECGI) is a tool developed to noninvasively identify onset of electric activation and propagation throughout the heart from body surface electrograms.5 From controlled torso-tank and animal experiments6–8 to clinical validation in different rhythm disorders and substrates, previously published data support the potential applicability of ECGI in the clinical assessment of arrhythmias. Prior studies have shown that noninvasive ECGI electrophysiological findings well correlated with conventional …