Pappone Carlo, Brugada Josep, Vicedomini Gabriele, Ciconte Giuseppe, Manguso Francesco, Saviano Massimo, Vitale Raffaele, Cuko Amarild, Giannelli Luigi, Calovic Zarko, Conti Manuel, Pozzi Paolo, Natalizia Andrea, Crisà Simonetta, Borrelli Valeria, Brugada Ramon, Sarquella-Brugada Georgia, Guazzi Marco, Frigiola Alessandro, Menicanti Lorenzo, Santinelli Vincenzo
From the Arrhythmology Department (C.P., G.V., G.C., F.M., M.S., R.V., A.C., L.G., Z.C., M.C., A.N., S.C., V.B., V.S.) and Cardiac Surgery Department (A.F., L.M.), IRCCS Policlinico San Donato, San Donato Milanese, Italy; Cardiology Department, Cardiovascular Institute, Hospital Clinic and IDIBAPS, Barcelona, Catalonia (J.B.); Cardiology Department, Hospital Trueta, Girona, Spain (R.B.); Department of Medical Sciences, University of Girona & IDIBGI, Spain (); Pediatric Arrhythmias, Electrophysiology and Sudden Death Unit, Cardiology Department, Hospital Sant Joan de Deu, Barcelona, Spain (G.S.-B.); and Cardiology Department, IRCCS Policlinico San Donato, University of Milan, Italy (M.G.).
Circ Arrhythm Electrophysiol. 2017 May;10(5):e005053. doi: 10.1161/CIRCEP.117.005053.
There is emerging evidence that localization and elimination of abnormal electric activity in the epicardial right ventricular outflow tract may be beneficial in patients with Brugada syndrome.
A total of 135 symptomatic Brugada syndrome patients having implantable cardiac defibrillator were enrolled: 63 (group 1) having documented ventricular tachycardia (VT)/ventricular fibrillation (VF) and Brugada syndrome-related symptoms, and 72 (group 2) having inducible VT/VF without ECG documentation at the time of symptoms. About 27 patients of group 1 experienced multiple implantable cardiac defibrillator shocks for recurrent VT/VF episodes. Three-dimensional maps before and after ajmaline determined the arrhythmogenic electrophysiological substrate (AES) as characterized by prolonged fragmented ventricular potentials. Primary end point was identification and elimination of AES leading to ECG pattern normalization and VT/VF noninducibility. Extensive areas of AES were found in the right ventricle epicardium, which were wider in group 1 (=0.007). AES increased after ajmaline in both groups (<0.001) and was larger in men (=0.008). The increase of type-1 ST-segment elevation correlated with AES expansion (=0.682, <0.001). Radiofrequency ablation eliminated AES leading to ECG normalization and VT/VF noninducibility in all patients. During a median follow-up of 10 months, the ECG remained normal even after ajmaline in all except 2 patients who underwent a repeated effective procedure for recurrent VF.
In Brugada syndrome, AES is commonly located in the right ventricle epicardium and ajmaline exposes its extent and distribution, which is correlated with the degree of coved ST-elevation. AES elimination by radiofrequency ablation results in ECG normalization and VT/VF noninducibility. Substrate-based ablation is effective in potentially eliminating the arrhythmic consequences of this genetic disease.
URL: https://clinicaltrials.gov. Unique identifier: NCT02641431.
越来越多的证据表明，定位并消除右心室流出道心外膜的异常电活动可能对布加综合征患者有益。
共纳入135例有症状且植入了植入式心脏除颤器的布加综合征患者：63例（第1组）有记录的室性心动过速（VT）/室颤（VF）及与布加综合征相关的症状，72例（第2组）在症状发作时可诱发VT/VF但无心电图记录。第1组约27例患者因反复发生的VT/VF发作而经历多次植入式心脏除颤器电击。阿义马林给药前后的三维标测确定了以心室碎裂电位延长为特征的致心律失常电生理基质（AES）。主要终点是识别并消除导致心电图模式正常化及VT/VF不可诱发性的AES。在右心室心外膜发现了广泛的AES区域，第1组的区域更宽（=0.007）。两组患者在阿义马林给药后AES均增加（<0.001），男性患者的AES更大（=0.008）。1型ST段抬高的增加与AES扩展相关（=0.682，<0.001）。射频消融消除了AES，使所有患者的心电图正常化且VT/VF不可诱发。在中位随访10个月期间，除2例因反复VF接受重复有效治疗的患者外，所有患者即使在阿义马林给药后心电图仍保持正常。
在布加综合征中，AES通常位于右心室心外膜，阿义马林可显示其范围和分布，这与穹窿形ST段抬高程度相关。通过射频消融消除AES可使心电图正常化且VT/VF不可诱发。基于基质的消融在潜在消除这种遗传性疾病的心律失常后果方面是有效的。
