Document Detail

Adenosine-Insensitive Right Ventricular Tachycardia: Novel Variant of Idiopathic Right Ventricular Outflow Tract Tachycardia.
MedLine Citation:
PMID:  24931634     Owner:  NLM     Status:  Publisher    
BACKGROUND: A hallmark of idiopathic right ventricular outflow tract (RVOT) tachycardia is its sensitivity to adenosine (ADO), which is consistent with a triggered mechanism. We have identified a novel group of patients with ADO-insensitive, non-reentrant RVOT tachycardia.
OBJECTIVE: To identify the clinical and electrophysiologic characteristics of ADO-insensitive RVOT tachycardia.
METHODS: The response of ventricular tachycardia (VT) to ADO was evaluated in 46 consecutive patients with inducible sustained idiopathic RVOT tachycardia. The clinical and electrophysiologic characteristics of patients with ADO-insensitive RVOT tachycardia were compared to patients with ADO-sensitive VT and arrhythmogenic right ventricular cardiomyopathy (ARVC)-associated VT.
RESULTS: Sustained RVOT tachycardia terminated with ADO in 41 (89%) patients while 5 (11%) patients had ADO-insensitive VT. The electrophysiology study findings of ADO-sensitive and ADO-insensitive RVOT patients were similar. Compared to a group of 10 ARVC patients, ADO-insensitive RVOT patients had no ARVC-associated ECG or right ventricular morphologic findings, as well as fewer inducible VT morphologies. Analysis of myocardial biopsies at VT origin sites from 3 of the 5 ADO-insensitive patients demonstrated somatic mutations in the A1 ADO receptor (A1AR R296C) in one patient and in the inhibitory G protein, (Gαi2 F200L) in another patient previously described. These mutations were not identified at remote myocardial sites. Over median follow-up of 4.8 years, no ADO-insensitive patient developed an ARVC phenotype.
CONCLUSIONS: Although most forms of idiopathic RVOT tachycardia are characterized by ADO-sensitivity, we describe a variant of ADO-insensitive VT which in some cases can be linked to somatic myocardial mutations involving the A1AR-associated cAMP-mediated pathway.
Jim W Cheung; James E Ip; Ravi K Yarlagadda; Christopher F Liu; George Thomas; Linna Xu; David Wilkes; Steven M Markowitz; Bruce B Lerman
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-6-12
Journal Detail:
Title:  Heart rhythm : the official journal of the Heart Rhythm Society     Volume:  -     ISSN:  1556-3871     ISO Abbreviation:  Heart Rhythm     Publication Date:  2014 Jun 
Date Detail:
Created Date:  2014-6-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101200317     Medline TA:  Heart Rhythm     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2014. Published by Elsevier Inc.
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