Document Detail

Mechanisms of sustained ventricular tachycardia in myotonic dystrophy: implications for catheter ablation.
MedLine Citation:
PMID:  9714111     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Ventricular arrhythmias have been documented and linked to the high incidence of sudden death seen in patients with myotonic dystrophy. However, their precise mechanism is unknown, and their definitive therapy remains to be established. METHODS AND RESULTS: We studied 6 consecutive patients with myotonic dystrophy and sustained ventricular tachycardia by means of cardiac electrophysiological testing. Particular attention was paid to establish whether bundle-branch reentry was the tachycardia mechanism, and when such was the case, radiofrequency catheter ablation of either the right or left bundle branch was performed. Clinical tachycardia was inducible in all patients and had a bundle-branch reentrant mechanism. In 1 patient, 2 other morphologies of sustained tachycardia were also inducible, neither of which had ever been clinically documented, and both had a bundle-branch reentrant mechanism. Ventricular tachycardia was no longer inducible after bundle-branch ablation, except for a nonclinically documented and nonsustained ventricular tachycardia in the only patient who had apparent structural heart disease. CONCLUSIONS: A high clinical suspicion of bundle-branch reentrant tachycardia is justified in patients with myotonic dystrophy who exhibit wide QRS complex tachycardia or tachycardia-related symptoms. Because catheter ablation will easily and effectively abolish bundle-branch reentrant tachycardia, myotonic dystrophy should always be considered in patients with sustained ventricular tachycardia. This is especially true if no apparent heart disease is found.
J L Merino; J R Carmona; I Fernández-Lozano; R Peinado; N Basterra; J A Sobrino
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Circulation     Volume:  98     ISSN:  0009-7322     ISO Abbreviation:  Circulation     Publication Date:  1998 Aug 
Date Detail:
Created Date:  1998-09-15     Completed Date:  1998-09-15     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  541-6     Citation Subset:  AIM; IM    
Department of Cardiology, Hospital General La Paz, Universidad Autónoma, Madrid, Spain.
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MeSH Terms
Catheter Ablation*
Defibrillators, Implantable
Middle Aged
Myotonic Dystrophy / complications*
Pacemaker, Artificial
Tachycardia, Ventricular / diagnosis,  etiology*,  surgery*
Treatment Outcome

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