Arrhythmogenic right ventricular cardiomyopathy: diverse substrate characteristics and ablation outcome

Wen Han Cheng, Fa Po Chung*, Yenn Jiang Lin, Li Wei Lo, Shih Lin Chang, Yu Feng Hu, Ta Chuan Tuan, Tze Fan Chao, Jo Nan Liao, Chin Yu Lin, Ting Yung Chang, Ling Kuo, Cheng I. Wu, Chih Min Liu, Shin Huei Liu, Shih Ann Chen

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

5 Scopus citations

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy caused by defective desmosomal proteins. The typical histopathological finding of ARVC is characterized by progressive fibrofatty infiltration of the right ventricle due to the dysfunction of cellular adhesion molecules, thus, developing arrhythmogenic substrates responsible for the clinical manifestation of ventricular tachycardia/fibrillation (VT/VF). Current guidelines recommend implantable cardiac defibrillator (ICD) implantation to prevent sudden cardiac death (SCD) in ARVC, especially for those experiencing VT/VF or aborted SCD, while antiarrhythmic drugs, despite their modest effectiveness and several undesirable adverse effects, are frequently used for those experiencing episodes of ICD interventions. Given the advances in mapping and ablation technologies, catheter ablation has been implemented to eliminate drug-refractory VT in ARVC. A better understanding of the pathogenesis, underlying arrhythmogenic substrates, and putative VT isthmus in ARVC contributes to a significant improvement in ablation outcomes through comprehensive endocardial and epicardial approaches. Regardless of ablation strategies, there is a diversity of arrhythmogenic substrates in ARVC, which could partly explain the nonuniform ablation outcome and long-term recurrences and reflect the role of potential factors in the modification of disease progression and triggering of arrhythmic events.

Original languageEnglish
Pages (from-to)1295-1309
Number of pages15
JournalReviews in Cardiovascular Medicine
Volume22
Issue number4
DOIs
StatePublished - 22 Dec 2021

Keywords

  • Arrhythmogenic right ventricular cardiomyopathy
  • Epicardial approach
  • Substrates
  • Ventricular fibrillation
  • Ventricular tachycardia

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