Chokr Muhieddine Omar, Filho Haroldo Heitor Ribeiro, Coello Roberto Alvarez, Kulchetscki Rodrigo Melo, Moura Lucas, Darrieux Francisco, Hachul Denise T, Sacilotto Luciana, Wu Tan C, Vandoni Pedro M, Souza Italo B, Linhares Pedro, Pisani Cristiano F, Hardy Carina A, Scanavacca Mauricio I
Arrhythmia Unit, Heart Institute, University of Sao Paulo Medical School, são Paulo, Brazil, Brazil.
J Cardiovasc Electrophysiol. 2025 Sep;36(9):2140-2150. doi: 10.1111/jce.16777. Epub 2025 Jul 1.
Bundle branch reentrant ventricular tachycardia (BBR-VT) is a rare form of VT occurring in patients with structural heart disease (SHD). Rarely, it can also occur in the absence of SHD. Understanding its clinical and electrophysiologic (EP) properties and outcomes post-catheter ablation (CA) is crucial.
We present a series of patients with bundle branch reentrant ventricular tachycardia (BBR-VT) and structurally normal hearts, comparing their clinical and electrophysiological characteristics, as well as long-term outcomes, with those of patients with the classic form of BBR-VT, in whom the arrhythmia was induced in the presence of ventricular dysfunction.
All cases of BBR-VT diagnosed during electrophysiological (EP) studies in our lab were evaluated. Clinical characteristics and EP findings were described.
Sixteen patients (12/16 male; mean age 50 ± 21 years) with BBR-VT were studied from 2009 to 2020, with a mean follow-up of 70 ± 16 months. Notably, nearly half (7/16; 43%) had no structural heart disease (SHD). Among these, three had myotonic dystrophy, two had SCN5A mutations, one had ajmaline-induced BBR-VT, and one had idiopathic BBR-VT. BBR-VT was induced with a mean cycle length of 322 ± 22 ms and was well tolerated in 10/16 patients. Right bundle branch catheter ablation was performed using an 8 mm solid-tip catheter, leading to an HV interval increase from 72 ± 9 ms to 100 ± 23 ms. One patient developed total AV block. Postprocedure, device implantation was required in 13/16 patients (dual-chamber pacemaker: 10; ICD: 3). Notably, no patient experienced VT recurrence during follow-up.
BBR-VT is traditionally associated with dilated cardiomyopathy, but it can also occur in structurally normal hearts. Myotonic dystrophy and SCN5A mutations may underlie some cases. Right bundle branch ablation is an effective treatment, though device implantation is usually required.
束支折返性室性心动过速(BBR-VT)是一种发生于结构性心脏病(SHD)患者的罕见室性心动过速形式。极少数情况下,它也可在无结构性心脏病时发生。了解其临床和电生理(EP)特性以及导管消融(CA)后的结局至关重要。
我们报告了一系列患有束支折返性室性心动过速(BBR-VT)且心脏结构正常的患者,将他们的临床和电生理特征以及长期结局与经典形式的BBR-VT患者(即在存在心室功能障碍时诱发心律失常的患者)进行比较。
对我们实验室在电生理(EP)研究期间诊断出的所有BBR-VT病例进行评估。描述临床特征和EP检查结果。
2009年至2020年对16例患有BBR-VT的患者(12/16为男性;平均年龄50±21岁)进行了研究,平均随访70±16个月。值得注意的是,近一半(7/16;43%)患者无结构性心脏病(SHD)。其中,3例患有强直性肌营养不良,2例有SCN5A突变,1例有阿义马林诱发的BBR-VT,1例为特发性BBR-VT。诱发BBR-VT的平均周期长度为322±22毫秒,10/16患者耐受性良好。使用8毫米实心尖端导管进行右束支导管消融,导致HV间期从72±9毫秒增加到100±23毫秒。1例患者发生完全性房室传导阻滞。术后,13/16患者需要植入装置(双腔起搏器:10例;植入式心脏复律除颤器:3例)。值得注意的是,随访期间无患者发生室性心动过速复发。
传统上BBR-VT与扩张型心肌病相关,但也可发生于心脏结构正常的患者。强直性肌营养不良和SCN5A突变可能是某些病例的潜在病因。右束支消融是一种有效的治疗方法,尽管通常需要植入装置。