Electroanatomic mapping of arrhythmogenic right ventricular dysplasia

Objectives We tested the hypothesis that spatial association of low-amplitude intracardiac electrograms can identify the presence, location and extent of dysplastic regions in arrhythmogenic right ventricular dysplasia (ARVD). Background Arrhythmogenic right ventricular dysplasia is a right ventricular (RV) cardiomyopathy characterized pathologically by fibrofatty infiltration and clinically by a spectrum of arrhythmias, sudden cardiac death and RV failure. Diagnosis of ARVD still remains a clinical challenge. Methods A three-dimensional electroanatomic mapping technique was used to map the RV of two groups of patients: 1) those with ARVD presenting with typical clinical, electrocardiographic and echocardiographic or magnetic resonance imaging (MRI) findings; and 2) those with structurally normal ventricles. Results The dysfunctional RV area could be identified only in the first group and was characterized by the presence of discrete areas of abnormally low-amplitude electrograms. Hence, the normal voltage values observed in the control group (unipolar: 11.9 ± 0.3 mV; bipolar: 4.6 ± 0.2 mV [mean ± SEM]) and in the nonaffected zones in the ARVD group (unipolar: 10.4 ± 0.2 mV; bipolar: 4.6 ± 0.2 mV) were reduced significantly (p < 0.05) in the dysplastic areas (unipolar: 3.3 ± 0.1 mV; bipolar: 0.5 ± 0.1 mV). The pathologic process mainly involved the RV anterolateral free wall, apex and inflow and outflow tracts and ranged from patchy areas to uniform and extensive involvement. Concordance between electroanatomic findings and MRI or echocardiographic findings was noted in all patients. Conclusions The pathologic substrate in ARVD can be identified by spatial association of low-amplitude endocardial electrograms, reflecting replaced myocardial tissue. The ability to accurately identify the presence, location and extent of the pathologic substrate may have important diagnostic, prognostic and therapeutic implications.