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Catheter ablation of frequent monomorphic ventricular arrhythmias in Andersen-Tawil syndrome: case report and focused literature review

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Abstract

Background/purpose

Andersen-Tawil syndrome type 1 is a rare autosomal dominant disease caused by a KCNJ2 gene mutation and clinically characterized by dysmorphic features, periodic muscular paralysis, and frequent ventricular arrhythmias (VAs). Although polymorphic and bidirectional ventricular tachycardias are prevalent, PVCs are the most frequent VAs. In addition, a “dominant” morphology with RBBB pattern associated with either superior or inferior axis is seen in most of the patients. Due to the limited efficacy of most antiarrhythmic drugs, catheter ablation (CA) is an alternative in patients with monomorphic VAs. Based on our experience, we aimed to review the arrhythmogenic mechanisms and substrates for VAs, and we analyzed the potential reasons for CA failure in this group of patients.

Methods

Case report and focused literature review.

Results

Catheter ablation has been reported to be unsuccessful in all of the few cases published so far. Most of the information suggests that VAs are mainly originated from the left ventricle and probably in the Purkinje network. Although identifying well-established and accepted mapping criteria for successful ablation of a monomorphic ventricular arrhythmia, papillary muscles seem not to be the right target.

Conclusions

More research is needed to understand better the precise mechanism and site of origin of VAs in Andersen-Tawil syndrome patients with this particular “dominant” monomorphic ventricular pattern to establish the potential role of CA.

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Acknowledgements

The authors would like to thank Juan Pablo Fabris for his contribution with clinical information to the case.

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Authors and Affiliations

  1. Electrophysiology Service, Hospital Privado del Sur, Bahía Blanca, Argentina

    Roberto Keegan, Leonardo Onetto, Franco Gregorietti & Ricardo Urruti

  2. Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA

    Luigi Di Biase

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  1. Roberto Keegan
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Correspondence to Roberto Keegan.

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Supplementary file1 (MP4 244 KB) ICE imaging during RF delivery at the posteromedial papillary muscle

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Keegan, R., Onetto, L., Gregorietti, F. et al. Catheter ablation of frequent monomorphic ventricular arrhythmias in Andersen-Tawil syndrome: case report and focused literature review. J Interv Card Electrophysiol 66, 729–736 (2023). https://doi.org/10.1007/s10840-021-01077-w

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