Leshem Eran, Rahkovich Michael, Mazo Anna, Suleiman Mahmoud, Blich Miri, Laish-Farkash Avishag, Konstantino Yuval, Fogelman Rami, Strasberg Boris, Geist Michael, Chetboun Israel, Swissa Moshe, Ilan Michael, Glick Aharon, Michowitz Yoav, Rosso Raphael, Glikson Michael, Belhassen Bernard
Department of Cardiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
Cardiovascular Institute, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.
Isr Med Assoc J. 2018 May;20(5):269-276.
Limited information exists about detailed clinical characteristics and management of the small subset of Brugada syndrome (BrS) patients who had an arrhythmic event (AE).
To conduct the first nationwide survey focused on BrS patients with documented AE.
Israeli electrophysiology units participated if they had treated BrS patients who had cardiac arrest (CA) (lethal/aborted; group 1) or experienced appropriate therapy for tachyarrhythmias after prophylactic implantable cardioverter defibrillator (ICD) implantation (group 2).
The cohort comprised 31 patients: 25 in group 1, 6 in group 2. Group 1: 96% male, mean CA age 38 years (range 13-84). Nine patients (36%) presented with arrhythmic storm and three had a lethal outcome; 17 (68%) had spontaneous type 1 Brugada electrocardiography (ECG). An electrophysiology study (EPS) was performed on 11 patients with inducible ventricular fibrillation (VF) in 10, which was prevented by quinidine in 9/10 patients. During follow-up (143 ± 119 months) eight patients experienced appropriate shocks, none while on quinidine. Group 2: all male, age 30-53 years; 4/6 patients had familial history of sudden death age < 50 years. Five patients had spontaneous type 1 Brugada ECG and four were asymptomatic at ICD implantation. EPS was performed in four patients with inducible VF in three. During long-term follow-up, five patients received ≥ 1 appropriate shocks, one had ATP for sustained VT (none taking quinidine). No AE recurred in patients subsequently treated with quinidine.
CA from BrS is apparently a rare occurrence on a national scale and no AE occurred in any patient treated with quinidine.
关于一小部分发生心律失常事件（AE）的 Brugada 综合征（BrS）患者的详细临床特征及治疗的信息有限。
开展首次针对有记录 AE 的 BrS 患者的全国性调查。
若以色列的电生理科室治疗过发生心脏骤停（CA）（致死性/非致死性；第 1 组）或在预防性植入式心律转复除颤器（ICD）植入后因室性快速心律失常接受过适当治疗的 BrS 患者（第 2 组），则参与调查。
该队列包括 31 名患者：第 1 组 25 名，第 2 组 6 名。第 1 组：男性占 96%，CA 平均年龄 38 岁（范围 13 - 84 岁）。9 名患者（36%）出现心律失常风暴，3 例死亡；17 例（68%）有自发 1 型 Brugada 心电图（ECG）。对 11 例可诱发出室颤（VF）的患者进行了电生理检查（EPS），其中 10 例可诱发，9/10 例患者的 VF 被奎尼丁预防。在随访期间（143 ± 119 个月），8 例患者接受了适当的电击，服用奎尼丁期间无电击发生。第 组：均为男性，年龄 30 - 53 岁；4/6 例患者有家族性猝死病史，年龄 < 50 岁。5 例患者有自发 1 型 Brugada ECG，4 例在 ICD 植入时无症状。对 4 例可诱发出 VF 的患者进行了 EPS，其中 3 例可诱发。在长期随访中，5 例患者接受了≥1 次适当的电击，1 例因持续性室速接受了 ATP 治疗（均未服用奎尼丁）。随后接受奎尼丁治疗的患者未再发生 AE。
在全国范围内，BrS 导致的 CA 显然很少见，服用奎尼丁的患者均未发生 AE。
