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Catheter ablation of frequent monomorphic ventricular arrhythmias in Andersen-Tawil syndrome: case report and focused literature review.
Keegan, Roberto; Onetto, Leonardo; Gregorietti, Franco; Urruti, Ricardo; Di Biase, Luigi.
Afiliação
  • Keegan R; Electrophysiology Service, Hospital Privado del Sur, Bahía Blanca, Argentina. robertokeegan@gmail.com.
  • Onetto L; Electrophysiology Service, Hospital Privado del Sur, Bahía Blanca, Argentina.
  • Gregorietti F; Electrophysiology Service, Hospital Privado del Sur, Bahía Blanca, Argentina.
  • Urruti R; Electrophysiology Service, Hospital Privado del Sur, Bahía Blanca, Argentina.
  • Di Biase L; Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY, USA.
J Interv Card Electrophysiol ; 66(3): 729-736, 2023 Apr.
Article em En | MEDLINE | ID: mdl-34665385
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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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Taquicardia Ventricular / Ablação por Cateter / Complexos Ventriculares Prematuros / Síndrome de Andersen Tipo de estudo: Prognostic_studies Limite: Humans Idioma: En Revista: J Interv Card Electrophysiol Assunto da revista: CARDIOLOGIA Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Argentina País de publicação: Holanda
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Taquicardia Ventricular / Ablação por Cateter / Complexos Ventriculares Prematuros / Síndrome de Andersen Tipo de estudo: Prognostic_studies Limite: Humans Idioma: En Revista: J Interv Card Electrophysiol Assunto da revista: CARDIOLOGIA Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Argentina País de publicação: Holanda