Document Detail


Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death.
MedLine Citation:
PMID:  12482795     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To investigate the clinical outcome, ECG characteristics, and optimal treatment of catecholaminergic polymorphic ventricular tachycardia (CPVT), a malignant and rare ventricular tachycardia.
PATIENTS AND METHODS: Questionnaire responses and ECGs of 29 patients with CPVT were evaluated. Mean (SD) age of onset was 10.3 (6.1) years.
RESULTS: The initial CPVT manifestations were syncope (79%), cardiac arrest (7%), and a family history (14%). ECGs showed sinus bradycardia and a normal QTc. Mean heart rate during CPVT was 192 (30) beats/min. Most cases were non-sustained (72%), but 21% were sustained and 7% were associated with ventricular fibrillation. The morphology of CPVT was polymorphic (62%), polymorphic and bidirectional (21%), bidirectional (10%), or polymorphic with ventricular fibrillation (7%). There was 100% inducement of CPVT by exercise, 75% by catecholamine infusion, and none by programmed stimulation. No late potential was recorded. Onset was in the right ventricular outflow tract in more than half the cases. During a follow up of 6.8 (4.9) years, sudden death occurred in 24% of the patients, 7% of whom had anoxic brain damage. Autosomal dominant inheritance was seen in 8% of the patients' families. beta Blockers completely controlled CPVT in only 31% of cases. Calcium antagonists partially suppressed CPVT in autosomal dominant cases.
CONCLUSIONS: CPVT may arise in certain distinct areas but the prognosis is poor. The onset of CPVT may be an indication for an implanted cardioverter-defibrillator.
Authors:
N Sumitomo; K Harada; M Nagashima; T Yasuda; Y Nakamura; Y Aragaki; A Saito; K Kurosaki; K Jouo; M Koujiro; S Konishi; S Matsuoka; T Oono; S Hayakawa; M Miura; H Ushinohama; T Shibata; I Niimura
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Heart (British Cardiac Society)     Volume:  89     ISSN:  1468-201X     ISO Abbreviation:  Heart     Publication Date:  2003 Jan 
Date Detail:
Created Date:  2002-12-16     Completed Date:  2003-01-31     Revised Date:  2013-06-09    
Medline Journal Info:
Nlm Unique ID:  9602087     Medline TA:  Heart     Country:  England    
Other Details:
Languages:  eng     Pagination:  66-70     Citation Subset:  AIM; IM    
Affiliation:
Department of Paediatrics, Nihon University School of Medicine, Itabashi, Tokyo, Japan. jt9n-smtm@asahi-net.or.jp
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adrenergic beta-Antagonists / therapeutic use
Adult
Anti-Arrhythmia Agents / therapeutic use
Catecholamines / genetics*
Catheter Ablation / methods
Child
Child, Preschool
Death, Sudden, Cardiac / prevention & control*
Echocardiography
Electrocardiography
Female
Heart Rate
Humans
Male
Polymorphism, Genetic
Survival Rate
Tachycardia, Ventricular / mortality,  physiopathology*,  therapy*
Treatment Outcome
Verapamil / therapeutic use
Chemical
Reg. No./Substance:
0/Adrenergic beta-Antagonists; 0/Anti-Arrhythmia Agents; 0/Catecholamines; 52-53-9/Verapamil
Comments/Corrections

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