Monaco Cinzia, Benali Karim, Cespon-Fernandez Maria, Bouteiller Xavier, Del Monte Alvise, Pannone Luigi, Surget Elodie, Mascia Giuseppe, Notarstefano Pasquale, Menè Roberto, Kneizeh Kinan, Yokoyama Masaaki, Arnaud Marine, Vlachos Konstantinos, Nakasone Kazutaka, Verhaeghe Laurens, Fitzgerald John L, Plant Allan, Della Rocca Domenico, Cathala Aude, Samson Sophie, Sieira Juan, Tixier Romain, Duchateau Josselin, Pambrun Thomas, Conte Giulio, Chierchia Gian Battista, Hocini Meleze, Porto Italo, Derval Nicolas, Mazzone Patrizio, Sacher Frederic, Jaïs Pierre, Brugada Pedro, de Asmundis Carlo, Haïssaguerre Michel
Cardiology Hospital Haut Lévêque, CHU Bordeaux, Pessac, France; IHU LIRYC (Cardiac electrophysiology and modeling), CRCTB (Inserm u1045), University of Bordeaux, Bordeaux, France; Heart Rhythm Management Centre, Postgraduate Program in Cardiac Electrophysiology and Pacing, European Reference Networks, Guard-Heart, Vrije Universiteit Brussel, Universitair Ziekenhuis Brussel, Jette, Belgium.
Cardiology Hospital Haut Lévêque, CHU Bordeaux, Pessac, France; IHU LIRYC (Cardiac electrophysiology and modeling), CRCTB (Inserm u1045), University of Bordeaux, Bordeaux, France; Saint-Etienne University Hospital Center, Saint-Etienne, France.
Heart Rhythm. 2025 Jun 20. doi: 10.1016/j.hrthm.2025.06.025.
Brugada syndrome (BrS) is an arrhythmogenic disorder associated with sudden cardiac death primarily owing to ventricular fibrillation (VF). Monomorphic ventricular tachycardias (MVTs) are rarely reported, and little is known about their characteristics.
This study aimed to evaluate the characteristics of patients with BrS presenting with MVT compared with those with VF.
We performed a retrospective multicenter study of patients with BrS who received an implantable cardioverter-defibrillator (ICD). Inclusion criteria were (1) the initiation of ventricular arrhythmia on ICD recordings and (2) the absence of antiarrhythmic drugs or previous ventricular ablation. Arrhythmic events were classified as MVT or polymorphic ventricular tachycardia (PVT)/VF (both referred to as VF) according to the guidelines. We analyzed clinical data, characteristics related to arrhythmia initiation, and electroanatomic substrate.
Among 793 patients with BrS with ICDs (44.2 ± 17.7 years, 37.1% women), 54 met the inclusion criteria. ICD recordings showed a total of 47 VF episodes and 27 MVT episodes, overlapping in only 1 patient. Patients with MVT were older at first event (47.7 ± 13.4 vs 40.7 ± 12.6 years, P = .06), had higher QRS duration, more prevalence of syncope, and later recurrences after ICD implantation (median 51 vs 20 months, P = .018). Rapid rhythms (>100 beats/min) were more frequent before MVT than VF (48.1% vs 8.5%, P < .001) with a lower incidence of ectopy (22.2% vs 61.7, P = .001). Abnormal epicardial substrate was broader in patients with MVT in baseline conditions (11.3 ± 5.5 cm vs 6.8 ± 3.2 cm, P = .006).
VF/PVT and MVT in patients with BrS have distinct clinical presentations. MVT shows a later onset compared with VF/PVT and is associated with a broader substrate area.
Brugada综合征（BrS）是一种致心律失常性疾病，主要与因室颤（VF）导致的心脏性猝死相关。单形性室性心动过速（MVT）鲜有报道，其特征也鲜为人知。
本研究旨在评估与室颤患者相比，表现为MVT的BrS患者的特征。
我们对接受植入式心律转复除颤器（ICD）的BrS患者进行了一项回顾性多中心研究。纳入标准为：（1）ICD记录上出现室性心律失常；（2）未使用抗心律失常药物或既往未进行过室性消融。根据指南，心律失常事件分为MVT或多形性室性心动过速（PVT）/VF（两者均称为VF）。我们分析了临床数据、与心律失常起始相关的特征以及电解剖基质。
在793例植入ICD的BrS患者中（44.2±17.7岁，37.1%为女性），54例符合纳入标准。ICD记录显示共有47次VF发作和27次MVT发作，仅1例患者两者重叠。MVT患者首次发病时年龄较大（47.7±13.4岁对40.7±12.6岁，P = 0.06），QRS时限更长，晕厥患病率更高，ICD植入后复发更晚（中位数51个月对20个月，P = 0.018）。MVT发作前快速心律（>100次/分钟）比VF更常见（48.1%对8.5%，P < 0.001），早搏发生率更低（22.2%对61.7%，P = 0.001）。基线情况下，MVT患者的异常心外膜基质更广泛（11.3±5.5 cm对6.8±3.2 cm，P = 0.006）。
BrS患者的VF/PVT和MVT有不同的临床表现。与VF/PVT相比，MVT发病较晚，且与更广泛的基质区域相关。
