Published Online
on August 18, 2003

A more recent version of this article appeared on August 26, 2003

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Submitted on March 4, 2003
Revised on July 9, 2003
Accepted on July 10, 2003

Mapping and Ablation of Ventricular Fibrillation Associated With Long-QT and Brugada Syndromes

Michel Haïssaguerre MD^*, Fabrice Extramiana MD, Mélèze Hocini MD, Bruno Cauchemez MD, Pierre Jaïs MD, Jose Angel Cabrera MD, Geronimo Farre MD, Antoine Leenhardt MD, Prashanthan Sanders MBBS, Christophe Scavée MD, Li-Fern Hsu MBBS, Rukshen Weerasooriya MBBS, Dipen C. Shah MD, Robert Frank MD, Philippe Maury MD, Marc Delay MD, Stéphane Garrigue MD, and Jacques Clémenty MD

From the Hopital Cardiologique du Haut-Leveque, Bordeaux-Pessac, France.

^* To whom correspondence should be addressed. E-mail: jacques.clementy{at}pu.u-bordeaux2.fr.

Background--The long-QT and Brugada syndromes are important substrates of malignant ventricular arrhythmia. The feasibility of mapping and ablation of ventricular arrhythmias in these conditions has not been reported.

Methods and Results--Seven patients (4 men; age, 38±7 years; 4 with long-QT and 3 with Brugada syndrome) with episodes of ventricular fibrillation or polymorphic ventricular tachycardia and frequent isolated or repetitive premature beats were studied. These premature beats were observed to trigger ventricular arrhythmias and were localized by mapping the earliest endocardial activity. In 4 patients, premature beats originated from the peripheral right (1 Brugada) or left (3 long-QT) Purkinje conducting system and were associated with variable Purkinje-to-muscle conduction times (30 to 110 ms). In the remaining 3 patients, premature beats originated from the right ventricular outflow tract, being 25 to 40 ms ahead of the QRS. The accuracy of mapping was confirmed by acute elimination of premature beats after 12±6 minutes of radiofrequency applications. During a follow-up of 17±17 months using ambulatory monitoring and defibrillator memory interrogation, no patients had recurrence of symptomatic ventricular arrhythmia but 1 had persistent premature beats.

Conclusion--Triggers from the Purkinje arborization or the right ventricular outflow tract have a crucial role in initiating ventricular fibrillation associated with the long-QT and Brugada syndromes. These can be eliminated by focal radiofrequency ablation.

Key words: long-QT syndrome • death, sudden • ablation • arrhythmia

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