Heterogeneous distribution of substrates between the endocardium and epicardium promotes ventricular fibrillation in arrhythmogenic right ventricular dysplasia/cardiomyopathy

Chin Yu Lin, Yenn Jiang Lin*, Cheng Hung Li, Fa Po Chung, Men Tzung Lo, Chen Lin, Hsiang Chih Chang, Shih Lin Chang, Li Wei Lo, Yu Feng Hu, Yao Ting Chang, Chung Hsing Lin, Yun Yu Chen, Rohit Walia, Abigail Louise D. Te, Shinya Yamada, Tsu Juey Wu, Shih Ann Chen

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

19 Scopus citations


Aims Whether the distribution of scar in arrhythmogenic right ventricular cardiomyopathy (ARVC) plays a role in predicting different types of ventricular arrhythmias is unknown. This study aimed to investigate the prognostic value of scar distribution in patients with ARVC. Methods and results We studied 80 consecutive ARVC patients (46 men, mean age 47 ± 15 years) who underwent an electrophysiological study with ablation. Thirty-four patients receive both endocardial and epicardial mapping. Abnormal endocardial substrates and epicardial substrates were characterized. Three groups were defined according to the epicardial and endocardial scar gradient (<10%: transmural, 10-20%: intermediate, >20%: horizontal, as groups 1, 2, and 3, respectively). Sinus rhythm electrograms underwent a Hilbert-Huang spectral analysis and were displayed as 3D Simultaneous Amplitude Frequency Electrogram Transformation (SAFE-T) maps, which represented the arrhythmogenic potentials. The baseline characteristics were similar between the three groups. Group 3 patients had a higher incidence of fatal ventricular arrhythmias requiring defibrillation and cardiac arrest during the initial presentation despite having fewer premature ventricular complexes. A larger area of arrhythmogenic potentials in the epicardium was observed in patients with horizontal scar. The epicardial-endocardial scar gradient was independently associated with the occurrence of fatal ventricular arrhythmias after a multivariate adjustment. The total, ventricular tachycardia, and VF recurrent rates were higher in Group 3 during 38 ± 21 months of follow-up. Conclusion For ARVC, the epicardial substrate that extended in the horizontal plane rather than transmurally provided the arrhythmogenic substrate for a fatal ventricular arrhythmia circuit.

Original languageEnglish
Pages (from-to)501-511
Number of pages11
Issue number3
StatePublished - 1 Mar 2018


  • Electroanatomic mapping
  • Hilbert-Huang transform
  • Sudden cardiac death
  • Ventricular fibrillation
  • Ventricular tachycardia


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