心外膜基质消融治疗Brugada综合征患者1例

1病例资料患者,男,52岁,发生心源性猝死,当地医院抢救成功,体表心电图显示I型Brugada波,动态心电图捕捉到心室颤动,确诊为Brugada综合征。转我院行ICD植入术,术后1个月内又反复出现心室颤动及ICD放电,给予药物治疗效果差,遂在我院行射频消融术。术前心电图提示为典型的I型Brugada波。24h动态心电图无室性期前收缩,无室性心动过速。     

Electrical storm in Brugada syndrome successfully treated using isoprenaline.

A case of an electrical storm occurring in a patient implanted with a cardioverter-defibrillator for Brugada syndrome is reported. Recurrent ventricular fibrillation was initiated by short-coupled isolated monomorphic ventricular premature beats probably originating from the right ventricular outflow tract, associated with a manifest electrocardiographic pattern of Brugada syndrome. Infusion of atropine accelerated the heart rate but did not prevent ventricular fibrillation, however, low doses of isoprenaline quickly obviated any recurrence of ventricular fibrillation. This was associated with the disappearance of the short-coupled premature beats together with a normalization of the electrocardiographic pattern. Possible mechanisms are discussed according to the accepted pathophysiological hypothesis.     

Successful use of quinine in the treatment of electrical storm in a child with Brugada syndrome

A 10-year-old girl developed life-threatening recurrent polymorphic ventricular tachycardia following surgical closure of a simple secundum atrial septal defect. Post hoc analysis of a Holter recording suggested Brugada syndrome. After managing the acute phase, a dual chamber defibrillator was implanted. One week later she experienced VF storm, needing 96 appropriate shocks within a few hours. Quinidine, by virtue of its I(to) blocking property, is the only drug reported to be useful in managing VF storm in Brugada syndrome. Nonavailability of quinidine led us to try its diastereomer, intravenous quinine, which succeeded in controlling the ventricular tachycardia. Arrhythmia storm in the setting of ion channelopathy can be difficult to manage, and sometimes requires innovative therapies.     

Incessant monomorphic ventricular tachycardia during febrile illness in a patient with Brugada syndrome: fatal electrical storm.

A 55-year-old male with structurally normal heart presented with sustained monomorphic ventricular tachycardia (VT) and was cardioverted into sinus rhythm revealing a right bundle branch block pattern at baseline electrocardiography. Sustained monomorphic and nonsustained polymorphic VT were reproducibly inducible during electrophysiological study. During the diagnostic workup, the patient experienced fever due to hospital based pneumonia, which unmasked typical ST segment changes of Brugada syndrome. In the intensive care unit, fever became intractable leading to incessant monomorphic VT, which was resistant to all medical manoeuvers resulting in the patient's death.     

Short-term normalization of ventricular repolarization by transcatheter ablation in a patient with suspected Brugada Syndrome

We report a 30 year old male without structural heart disease who presented with recurrent nocturnal syncope and aborted sudden cardiac death. 12-lead ECG showed elevated ST in inferior leads and short coupled premature ventricular complexes (PVCs). Propafenone challenge suggested a diagnosis of an atypical Brugada syndrome. Two morphological types of PVCs and ventricular fibrillation (VF) were induced during propafenone challenge test. He underwent two ablation procedures in right ventricular inflow tract and left ventricular post-inferior septum region by pace-mapping, respectively. After ablation, VF could not be induced and the elevated ST segments normalized. Two subsequent propafenone challenge tests were also negative. Nonetheless, elevated ST segments and PVCs reappeared by 1 month follow-up. An implantable defibrillator was recommended, but the patient declined for financial reasons. Unfortunately, he suffered a sudden cardiac death at home 10 weeks post-ablation. These findings suggest that short-term normalization of ventricular repolarization possibly due to radiofrequency ablation may occur in Brugada syndrome. However, the transient nature of this finding suggests that it is not a reliable indicator of protection against sudden cardiac death.     

经导管射频消融心律转复除颤器植入后电风暴

目的报道3例心律转复除颤器（ICD）植入后抗心律失常药物治疗无效的室性心律失常电风暴患者经导管射频消融的结果。方法2名男性与1名女性患者,年龄为75、55、37岁,分别患有陈旧性前壁心肌梗死、致心律失常性右心室心肌病、左心室心肌病。均在ICD植入后发生抗心律失常药物治疗无效的电风暴。应用Carto电解剖标测系统引导盐水灌注射频导管标测和消融室性心动过速（VT）。对可标测VT（持续性、血流动力学稳定）行激动和拖带标测;对不可标测VT,则在基质标测的基础上行起搏标测和／或短时间的拖带标测。结果3例患者中共诱发出5种形态的VT,4种血流动力学较稳定VT和1种血流动力学不稳定VT。成功消融了所有形态的VT,抑制了电风暴的急性发作。消融后随访的6、19和36个月中,仅1例患者出现1次ICD放电。结论在电解剖标测的基础上,应用盐水灌注射频导管消融ICD植入后抗心律失常药物治疗无效的电风暴有很好的疗效。     

Brugada综合征快速心律失常的发病机理与射频消融治疗

目的探讨Brugada综合征ST-T抬高与快速心律失常的发病机理和射频消融治疗。方法选择体表心电图V1~V3相邻2个导联ST-T下斜或马鞍型抬高≥0.2mV,且有心律失常者按常规行心脏电生理检查和射频消融。结果38例患者,男31例,女7例,年龄38.27±13.91岁。17例有晕厥或黑矇,3例经历心肺复苏。23例为Ⅰ型Brugada图形,10例为Ⅱ型,5例为Ⅰ、Ⅱ、Ⅲ型交替,均合并不同类型的心脏传导异常。10例为单形(2例)或多形(8例)室性心动过速,1例为心室颤动电风暴,5例为室性与房性心律失常并存,5例为单纯室上性心动过速(4例为显性预激综合征),3例为室上性心动过速和心房扑动,14例为房性早搏、房性心动过速和心房颤动。射频消融即刻成功32例(成功率84%),失败6例,未发生并发症。消融成功者心律失常消失后即刻,ST-T抬高未见改变。而室性早搏、房性早搏伴差传、His束传导阻滞、间歇预激旁道、心室起搏与静脉滴注异丙肾上腺素可使ST-T抬高幅度改变或逆转。随访5.72±2.03年,1例失访,5例心律失常复发,1例猝死,3例安装心脏永久起搏器,1例安装心脏转复除颤器。结论Brugada综合征常见心脏传导异常,ST-T抬高继发于心脏传导系统疾病与除极顺序改变,而非原发心内、外膜复极离散与2相折返,射频消融可有效防治Brugada综合征的多种快速心律失常。     

Management of electrical storm of unstable ventricular tachycardia in post myocardial infarction patients: A single centre experience

ObjectiveThis is a case series of consecutive patients with past myocardial infarction presenting with Electrical Storm (ES) of unstable ventricular tachycardia (VT) treated by a protocol directed algorithm.MethodsManagement protocol involved treatment of reversible causes, ventilatory & hemodynamic support, administration of antiarrhythmic drugs (AAD) & maximally tolerated doses of beta-blockers, stellate ganglionectomy and Radiofrequency ablation (RFA) guided by Electro Anatomic Mapping (EAM). Patients were followed up periodically with review of device data logs.ResultsThere were 12 patients (mean age = 61.38 ± 6.48 years & mean LVEF = 31.92 ± 4.23%). Presentation was recurrent ICD shocks (n = 5) or VT (n = 7). All were mechanically ventilated. Reversible causes were identified in 4 patients and appropriately addressed. Totally 8 patients underwent endocardial substrate modification by EAM & RFA. Endocardial LV Voltage mapping demonstrated a mean scar area of 70.04 ± 17.63 sq.cm (27.04 ± 6.20% of mapped area). The electrograms targeted for ablation included late potentials, fractionated electrograms, double potentials and channels within the scar. Two patients had stellate ganglionectomy in addition. Ten patients (83.3%) survived to discharge, all of whom are alive at a follow up of 30.12 ± 19 months free of ES. VT free survival at end of follow up was 80%. No patient had hospitalization related to VT. Single episode of VT recurrence was seen in 2 patients at 7 months and 1 year of follow up respectively.ConclusionIn post myocardial infarction patients presenting with ES and unstable VT, a protocol driven approach involving substrate modification targeting abnormal electrograms improves outcomes.     

Role of endocardial ablation in eliminating an epicardial arrhythmogenic substrate in patients with Brugada syndrome

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Atrial fibrillation: Catheter ablation

Catheter ablation of atrial fibrillation (AF) has evolved dramatically over the last several years. The initial efforts in the catheter-based management of AF targeted the atrial substrate in an effort to mimic the maze procedure. After the pulmonary veins (PV) were shown to be critical in the initiation and perpetuation of AF, the focus then shifted to a trigger approach in which the PVs and other foci were targeted for ablation. The pendulum then appeared to swing back toward the substrate approach after it was shown that left atrial circumferential ablation afforded improved outcomes in patients with paroxysmal and persistent AF. It has become clear that there are several possible approaches in the catheter ablation of AF, each with its strengths and limitations. It is also becoming evident that not all patients will respond to a single ablation technique and that the ablation protocol is best tailored to suit the individual patient. This article strives to present an evidence-based review of the many techniques, and then offer a practical guide to the catheter ablation of AF.     

Electrical storm in systemic sclerosis: Inside the electroanatomic substrate

We report the case of a 63-year-old woman affected by a severe form of systemic scleroderma with pul-monary involvement(interstitial fibrosis diagnosed by biopsy and moderate pulmonary hypertension) and cardiac involvement(paroxysmal atrial fibrillation, right atrial flutter treated by catheter ablation, ventricular tachyarrhythmias, previous dual chamber implantable cardioverter defibrillator implant). Because of recurrent electrical storms refractory to iv antiarrhythmic drugs the patient was referred to our institution to undergo catheter ablation. During electrophysiological proce-dure a 3D shell of cardiac anatomy was created with intracardiac echocardiography pointing out a significant right ventricular dilatation with a complex aneurysmal lesion characterized by thin walls and irregular multiple trabeculae. A substrate-guided strategy of catheter ab-lation was accomplished leading to a complete electri-cal isolation of the aneurism and to the abolishment of all abnormal electrical activities. The use of advanced strategies of imaging together with electroanatomical mapping added important information to the complex arrhythmogenic substrate and improved efficacy and safety.