TY - JOUR
T1 - Catheter ablation of frequent monomorphic ventricular arrhythmias in Andersen-Tawil syndrome
T2 - case report and focused literature review
AU - Keegan, Roberto
AU - Onetto, Leonardo
AU - Gregorietti, Franco
AU - Urruti, Ricardo
AU - Di Biase, Luigi
N1 - Publisher Copyright:
© 2021, Springer Science+Business Media, LLC, part of Springer Nature.
PY - 2023/4
Y1 - 2023/4
N2 - 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.
AB - 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.
KW - Andersen-Tawil syndrome
KW - Catheter ablation
KW - Papillary muscles
KW - Purkinje network
KW - Ventricular arrhythmias
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U2 - 10.1007/s10840-021-01077-w
DO - 10.1007/s10840-021-01077-w
M3 - Review article
C2 - 34665385
AN - SCOPUS:85117234482
SN - 1383-875X
VL - 66
SP - 729
EP - 736
JO - Journal of Interventional Cardiac Electrophysiology
JF - Journal of Interventional Cardiac Electrophysiology
IS - 3
ER -