Document Detail

Arrhythmia Characterization and Long-term Outcomes in Catecholaminergic Polymorphic Ventricular Tachycardia.
MedLine Citation:
PMID:  21315846     Owner:  NLM     Status:  Publisher    
BACKGROUND: Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is characterized by adrenergically-induced ventricular tachycardia (VT) associated with syncope and sudden death. OBJECTIVES: (1) characterize arrhythmias associated with CPVT with respect to provocation by exercise and drugs, ECG characteristics, and association with long-term outcomes; (2) explore relation between age and clinical presentation. METHODS: Seventy patients from 16 families were evaluated with exercise and selective adrenaline challenge, and screened for RyR2 mutations. CPVT was diagnosed in probands with symptoms and stress- or adrenaline-provoked VT, or in asymptomatic relatives with provoked VT or RyR2 mutations. Patients were followed for recurrent syncope, VT and sudden death. RESULTS: Twenty-seven patients including 16 probands were identified (median age 35 years, 67% female). Presentation was cardiac arrest in 33%, syncope in 56%, and 11% were asymptomatic. Polymorphic or bidirectional VT was provoked with exercise in 63% and adrenaline in 82%. The initiating beat of VT was late-coupled and wide (coupling interval 418±42 msec; QRSd 131±17 msec), and QRS morphology suggested an outflow tract origin in 59%. During follow-up of 6.2±5.7 years, two patients died despite an ICD, four patients received ICD therapy for VT, and five patients had inappropriate therapy for SVT. Patients presenting with "late-onset" CPVT (age>21; n=10) were often female (80%), less likely to have RyR2 mutations (33%) and fatal events were not observed during follow-up (4.1±3.6 years). CONCLUSIONS: Ventricular arrhythmia in CPVT is often initiated from the outflow tract region. Despite β-blocker therapy and selective ICD implantation, breakthrough arrhythmias occur and may be associated with adverse outcomes.
Raymond W Sy; Michael H Gollob; George J Klein; Raymond Yee; Allan C Skanes; Lorne J Gula; Peter Leong-Sit; Robert M Gow; Martin S Green; David H Birnie; Andrew D Krahn
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-2-9
Journal Detail:
Title:  Heart rhythm : the official journal of the Heart Rhythm Society     Volume:  -     ISSN:  1556-3871     ISO Abbreviation:  -     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-2-14     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 © 2011. Published by Elsevier Inc.
University of Western Ontario.
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