Haïssaguerre Michel, Extramiana Fabrice, Hocini Mélèze, Cauchemez Bruno, Jaïs Pierre, Cabrera Jose Angel, Farré Jerónimo, Leenhardt Antoine, Sanders Prashanthan, Scavée Christophe, Hsu Li-Fern, Weerasooriya Rukshen, Shah Dipen C, Frank Robert, Maury Philippe, Delay Marc, Garrigue Stéphane, Clémenty Jacques
Hôpital Cardiologique du Haut-Lévêque, Avenue de Magellan, 33604 Bordeaux-Pessac, France.
Circulation. 2003 Aug 26;108(8):925-8. doi: 10.1161/01.CIR.0000088781.99943.95. Epub 2003 Aug 18.
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.
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.
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.
长QT综合征和Brugada综合征是恶性室性心律失常的重要基质。在这些情况下进行室性心律失常标测和消融的可行性尚未见报道。
研究了7例患者（4例男性；年龄38±7岁；4例长QT综合征，3例Brugada综合征），这些患者有室颤或多形性室性心动过速发作，以及频繁的孤立性或重复性早搏。观察到这些早搏可触发室性心律失常，并通过标测最早的心内膜活动进行定位。4例患者中，早搏起源于外周右（1例Brugada综合征）或左（3例长QT综合征）浦肯野传导系统，且与不同的浦肯野-心肌传导时间（30至110毫秒）相关。其余3例患者中，早搏起源于右心室流出道，比QRS提前25至40毫秒。在射频应用12±6分钟后早搏急性消除，证实了标测的准确性。在使用动态监测和除颤器内存询问进行17±17个月的随访期间，无患者出现有症状的室性心律失常复发，但1例患者有持续性早搏。
浦肯野分支或右心室流出道的触发因素在引发与长QT综合征和Brugada综合征相关的室颤中起关键作用。这些触发因素可通过局灶性射频消融消除。
