Disease-Specific Electrocardiographic Lead Positioning for Early Detection of Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by replacement of cardiomyocytes by fibrofatty tissue which can lead to ventricular arrhythmias, heart failure or sudden cardiac death. Genetic defects in desmosomal proteins, as plakophilin-2 (PKP2), are known to contribute to disease development. Current electrocardiographic (ECG) criteria for ARVC diagnosis only focus on right precordial leads, but sensitivity of current depolarization criteria is limited. This study aimed to identify additional depolarization criteria with most optimal lead configurations for early detection of ARVC in PKP2 pathogenic mutation carriers. In PKP2-positive ARVC patients (n=7), PKP2 pathogenic variant carriers (n=16) and control subjects without structural heart disease (n=9), 67-lead body surface potential maps (BSPM) were obtained. Terminal QRS-integrals were determined and quantitatively compared to controls using departure mapping. Significantly different terminal QRS-integrals were identified in lead 34 (conventional V3), 40 and 41 (conventional V4). To conclude, a clear distinction between ARVC patients, asymptomatic mutation carriers and healthy controls was observed.