Department of Cardiology, Isala Heart Center, Dr. Van Heesweg 2, 8025 AB, Zwolle, The Netherlands.
Academic Medical Center, Amsterdam, The Netherlands.
Clin Res Cardiol. 2020 May;109(5):560-569. doi: 10.1007/s00392-019-01540-9. Epub 2019 Sep 2.
In the past few years, promising results were described in targeting the arrhythmogenic substrate of the epicardial right ventricular outflow tract (RVOT) region in patients with Brugada syndrome (BrS). In this report, we describe our experience with endo- and epicardial substrate mapping and ablation in a series of highly symptomatic BrS patients.
This case series consists of seven patients with clinical BrS diagnosis who underwent catheter ablation in two Dutch hospitals (Isala hospital Zwolle; and Amsterdam University Medical Centre, location AMC, Amsterdam) and Hamad Heart Hospital in Qatar between 2013 and 2017. All patients had an ICD and recurrent ventricular arrhythmia (VA) episodes. All patients underwent endo-and epicardial mapping of the RVOT region. Elimination of all abnormal potentials and disappearance of BrS ECG pattern during the ablation procedure was the aimed endpoint.
The study group consisted of seven patients with mean age 45.6 ± 16.9 years. Five patients had SCN5A mutations. One patient was excluded from analysis, since ablation could not be performed due to a very large low-voltage area and was later diagnosed with arrhythmogenic right ventricular cardiomyopathy, associated with an SCN5A mutation. One patient underwent both endo- and epicardial ablation to eliminate VA. During a mean follow-up of 3.6 ± 1.5 years, 5/6 patients remained VA free with two patients continuing quinidine.
In patients with BrS and drug-refractory VA, ablation of the arrhythmogenic substrate in the RVOT region was associated with excellent long-term VA-free survival. The majority of these highly symptomatic BrS patients had an SCN5A mutation and also low-voltage areas epicardially.
在过去的几年中，针对 Brugada 综合征（BrS）患者心外膜右心室流出道（RVOT）区域的致心律失常基质的靶向治疗取得了令人鼓舞的结果。在本报告中，我们描述了在一系列有症状的 BrS 患者中进行心内膜和心外膜基质标测和消融的经验。
本病例系列包括七例在 2013 年至 2017 年期间在荷兰的两家医院（Zwolle 的 Isala 医院和阿姆斯特丹大学医学中心，阿姆斯特丹 AMC 院区）和卡塔尔的哈马德心脏医院接受导管消融的临床诊断为 BrS 的患者。所有患者均植入了 ICD 并发生反复性室性心律失常（VA）发作。所有患者均接受了 RVOT 区域的心内膜和心外膜标测。消融过程中消除所有异常电位并消除 BrS 心电图模式是我们的目标终点。
研究组由七名平均年龄为 45.6±16.9 岁的患者组成。五名患者存在 SCN5A 突变。一名患者由于存在非常大的低电压区域而被排除在分析之外，该患者随后被诊断为致心律失常性右心室心肌病，伴有 SCN5A 突变。一名患者同时进行了心内膜和心外膜消融以消除 VA。平均随访 3.6±1.5 年后，6/5 例患者 VA 持续消失，其中 2 例继续服用奎尼丁。
在 BrS 患者和药物难治性 VA 患者中，消融 RVOT 区域的致心律失常基质与良好的长期 VA 无复发生存相关。这些有症状的 BrS 患者中大多数存在 SCN5A 突变，并且心外膜也存在低电压区。
