Arrhythmogenic right ventricular cardiomyopathy: Electroarchitecture of the substrate

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by ventricular arrhythmias and fibrofatty replacement of the right ventricular (RV) myocardium. Although the RV is the predominant chamber involved, left ventricular (LV) involvement has also been documented. Electrophysiological correlations of histopathologic findings in nonischemic cardiomyopathy, and especially in ARVC, are limited. We report a case of ARVC that presented with ventricular tachycardia (VT) and diffuse fibrofatty involvement of both the RV and LV. A detailed analysis of the electrophysiological, cardiac imaging, and pathologic findings was performed, providing insights into the electroachitecture of the myocardium and electrical manifestations of the pathologic findings.