From the Arrhythmia Unit, Department of Cardiology, Hospital General La
Paz, Universidad Autónoma, Madrid, Spain (J.L.M., R.P., J.A.S.); the
Coronary Care and Arrhythmia Unit, Department of Cardiology, Hospital de
Navarra, Pamplona, Spain (J.R.C., N.B.); and the Arrhythmia Unit, Department
of Cardiology, Clínica Puerta de Hierro, Universidad Autónoma,
Madrid, Spain (I.F.-L.).
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.
© 1998 American Heart Association, Inc.
Clinical Investigation and Reports
Mechanisms of Sustained Ventricular Tachycardia in Myotonic Dystrophy
Implications for Catheter Ablation
Key Words: ablation • bundle-branch block • electrophysiology • myotonia atrophica • tachycardia
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