Work

Body surface potential mapping of ventricular depolarization and repolarization in phospholamban and plakophilin-2 cardiomyopathy

Year: 2025

Type: article

Abstract: Background Pathogenic variants in plakophilin -2 ( PKP2 ) and phospholamban ( PLN ) are associated with arrhythmogenic cardiomyopathy. Early disease detection is important to prevent adverse events. Body surface potential mapping (BSPM) may detect local electrical abnormalities earlier than the 12-lead electrocardiogram. Objective To determine abnormalities in R-, S- and T-wave amplitudes in PKP2 and PLN pathogenic variant carriers using BSPM. Methods Sixty-seven lead BSPM was performed in controls, PKP2 - and PLN -carriers. R-, S-, and T-wave amplitudes across all leads in controls were used as reference. Amplitudes of carriers exceeding these ranges were considered abnormal, and assessed across disease stages (pre-symptomatic, electrical and structural, as done previously). Follow-up BSPM (≥2 years) was performed in a subset of carriers. Results 152 subjects (40[27;54] years, 51% female); 40 controls and 112 carriers (53 PKP2 , 59 PLN ) were included. Amplitude abnormalities were most frequent in structural disease, predominantly in T-waves ( PKP2 : 20[10;29], PLN : 25[22;30] leads). Abnormalities in electrical disease were more prevalent in PLN -carriers than PKP2 -carriers (R-wave: 4[1;7] vs 13[8;16] leads, P =0.002; S-wave: 2[1;3] vs 4[3;12] leads, P <0.001; T-wave: 1[0;3] vs 20[16;28] leads, P <0.001). Pre-symptomatic carriers typically had abnormalities outside the 12-lead configuration. As disease progressed, abnormalities became more frequent and extended towards V1-V6. Follow-up BSPM (23 PKP2 , 16 PLN ), showed consistency in locations of abnormalities with increased frequency (maximal increase: 31%). Conclusion BSPM detected abnormal amplitudes within and beyond the 12-lead electrocardiogram, even in pre-symptomatic carriers. Follow-up BSPM suggests these abnormalities are associated with disease progression, highlighting the potential benefit of BSPM in early disease detection.

Source: Heart Rhythm O2

DOI: 10.1016/j.hroo.2025.09.027