Clinical outcomes of catheter ablation of ventricular tachycardia in patients with arrhythmogenic right ventricular cardiomyopathy: Insights from the Johns Hopkins ARVC Program

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三维标测指导致心律失常性右心室心肌病室性心动过速的射频消融

目的介绍致心律失常性右心室心肌病（ARVC）室性心动过速（室速）的三维标测方法及其消融策略。方法21例ARVC室速患者,因1—4种抗心律失常药物治疗无效,临床上呈反复发作、无休止发作或植入型心律转复除颤器（ICD）植入后频繁放电治疗,接受导管消融治疗。其中,男性19例,女性2例,平均年龄（32±12）岁。9例患者接受电解剖（Carto）标测,12例患者接受非接触标测（EnSite—Array）。在首先明确病变基质的基础上,通过激动标测、拖带标测及起搏标测,分析心动过速的起源、可能的传导径路及其出口以及它们与病变基质的关系。通常于心动过速的出口处及其周边行局灶消融,术中病变基质周边的延迟激动电位应一并消融。结果21例患者,2例呈无休止发作,1例患者表现为频繁室性早搏及加速性室性自主心律,余18例患者消融中共诱发出34种心动过速。所有心动过速均呈左束支阻滞形,平均心动过速周长为（289±68）ms。16例患者（28种室速）消融治疗即刻成功,3例患者（7种室速）部分成功,2例患者（2种室速）消融失败,即刻消融成功率76．2％。所有患者消融术后继续服用抗心律失常药物。平均随访6～30（1d±7）个月,成功患者中2例复发,其中1例再次消融成功;未达即刻成功的5例患者,经抗心律失常药物治疗后,均无室性心律失常事件发生,其中包括1例消融后植入ICD者。结论三维标测系统可首先明确ARVC患者的病变基质,在此基础上结合激动标测和心内各种电刺激技术,可直观显示心动过速的起源、缓慢传导区出口及折返环路,以此制定消融策略可成功治疗ARVC室速。心动过速起源于心肌深部或ARVC病变进展,是消融失败和复发的常见原因。     

Macroreentrant ventricular tachycardia mimicking focal ventricular tachycardia in a case with arrhythmogenic right ventricular cardiomyopathy

A 67-year-old man with ventricular tachycardia (VT) due to arrhythmogenic right ventricular cardiomyopathy (ARVC) underwent electrophysiologic testing. Electroanatomic mapping during the VT seemed to reveal a focal mechanism from near the tricuspid annulus (TA). Several radiofrequency applications delivered at the presumed focus resulted in termination but re-induction of the VT. Additional electroanatomic mapping underneath the TA led to the diagnosis of macroreentrant VT. Several RF applications targeting isolated and late potentials observed there during sinus rhythm eliminated the VT. In ARVC cases, detailed mapping underneath the TA should be performed to reveal the VT mechanism, resulting in suppressing VT recurrences. There was no financial support for this study.     

致心律失常性右室心肌病室性心动过速的射频消融治疗

目的评价致心律失常性右室心肌病(ARVC)室性心动过速(VT)射频消融的疗效。方法 4例ARVC患者,男3例,女1例,年龄27～62岁,均有反复头昏、心悸、晕厥或黑矇病史。4例患者症状发作时共出现6种形态的VT,频率为130～210次/分。在三维标测系统(CARTO或EnSite Array)指导下行VT消融治疗。结果 4例患者共行6次手术,其中3次采用CARTO系统,3次采用EnSiteArray系统指导。3例完成消融,随访2～19个月,3例患者均无猝死、晕厥或黑矇发生;2例术后一周内复发,但VT的频率减慢,药物能有效控制,术后5～6个月VT不再发作。另1例患者在放电消融过程中VT的频率加快,形态紊乱,蜕变为心室颤动,紧急电复律后转为窦性心律,终止手术。随访6个月,无VT发生。结论致心律失常性右室心肌病VT的射频消融治疗可改善病人的症状。     

Ventricular tachycardia mapping and ablation in arrhythmogenic right ventricular cardiomyopathy/dysplasia:Lessons Learned

Arrhythmogenic right ventricular cardiomyopathy/dysplasia(ARVC/D) is primarily believed to be an inherited cardiomyopathy that subsequently results in significant myocardial fibrosis. The arrhythmogenic consequences that result from the development of fibrosis are similar to other nonischemic cardiomyopathies, but the unique endocardial-epicardial disease process of ARVC/D requires a specialized approach for arrhythmia treatment in the electrophysiology laboratory. Although the association between ARVC/D and development of ventricular arrhythmias has become increasingly clear over the last 2 decades, our understanding of the arrhythmia mechanisms, underlying electrophysiologic substrate, and treatment strategies were significantly limited. Prospective studies performed in the electrophysiology laboratory allowed detailed characterization of the electrophysiologic and electroanatomic substrate underlying ventricular tachycardia in patients with ARVC/D. Thishas allowed clinician scientists to better characterize the arrhythmia mechanism and develop the necessary strategies to perform successful catheter ablation. Early in this experience, catheter ablation was considered a limited and largely unsuccessful treatment for patients experiencing painful and recurrent defibrillator therapy. Through our increased understanding of the disease process, catheter ablation has evolved to become an effective and preferred therapy for a majority of these patients. Our understanding of the disease and necessary approaches to provide successful treatment continues to evolve as the clinical experience grows. This article will review these important insights from the electrophysiology laboratory and how application of this knowledge has facilitated the development of a methodical approach to successfully perform ventricular tachycardia ablation in patients with ARVC/D.     

Ablation strategies for arrhythmogenic right ventricular cardiomyopathy:a systematic review and meta-analysis

BackgroundCatheter ablation for ventricular tachycardia (VT) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) has significantly evolved over the past decade. However, different ablation strategies showed inconsistency in acute and long-term outcomes.MethodsWe searched the databases of Medline, Embase and Cochrane Library through October 17, 2019 for studies describing the clinical outcomes of VT ablation in ARVC. Data including VT recurrence, all-cause mortality, acute procedural efficacy and major procedural complications were extracted. A meta-analysis with trial sequential analysis was further performed in comparative studies of endo-epicardial versus endocardial-only ablation.ResultsA total of 24 studies with 717 participants were enrolled. The literatures of epicardial ablation were mainly published after 2010 with total ICD implantation of 73.7%, acute efficacy of 89.8%, major complication of 5.2%, follow-up of 28.9 months, VT freedom of 75.3%, all-cause mortality of 1.1% and heart transplantation of 0.6%. Meta-analysis of 10 comparative studies revealed that compared with endocardial-only approach, epicardial ablation significantly decreased VT recurrence (OR: 0.50; 95% CI: 0.30-0.85; P = 0.010), but somehow increased major procedural complications (OR: 4.64; 95% CI: 1.28-16.92; P= 0.02), with not evident improvement of acute efficacy (OR: 2.74; 95% CI: 0.98-7.65; P = 0.051) or all-cause mortality (OR: 0.87; 95% CI: 0.09-8.31; P = 0.90).ConclusionCatheter ablation for VT in ARVC is feasible and effective. Epicardial ablation is associated with better long-term VT freedom, but with more major complications and unremarkable survival or acute efficacy benefit.     

Bundle branch reentry ventricular tachycardia in arrhythmogenic right ventricular dysplasia

A 42-year-old male had history of recurrent palpitation and was documented to have wide QRS tachycardia. Magnetic resonance imaging angiogram showed evidence of arrhythmogenic right ventricular dysplasia and severe right ventricular dysfunction. Electrophysiology study showed evidence of bundle branch reentry ventricular tachycardia. It was successfully treated by radiofrequency ablation of right bundle branch. This is probably the first case of bundle branch reentry as a mechanism for ventricular tachycardia in a case of arrhythmogenic right ventricular dysplasia.     

体表心电图特征对特发性右心室流出道室性心动过速和室性早搏起源点的定位价值

目的 起源于右心室流出道(RVOT)不同位点的室性心动过速(VT)具有相应的心电图表现,本研究旨在摸索一种相对简单的根据体表心电图进行定位的方法 .方法 将RVOT分为游离壁和间隔而两大区,其中间隔面又分为9个区域.共320例RVOT-VT患者中,对213例既往消融成功患者的靶点与体表12导联心电图中QRS波形态之间的关系进行分析,并在消融前前瞻性地对另外107例患者的消融靶点进行预测,以检验其定位价值.结果 I导联对RVOT起源的VT有特殊的定位价值.在间隔面前部起源时,I导联以负向波为主,多为QS、Qr及rS型,随着起源点从前向后、从上向下,R波逐渐升高,其中起源于间隔侧中带(2、5、8区)时,以"M"型居多,在后壁时则表现为R波且有切迹.游离壁起源者的QRS时限明显延长,I和aVL导联的R波较间隔起源者高,而下壁导联的R波均较间隔的低(P<0.05).在前瞻性分析中,这些参数的敏感度、特异度、阳性和阴性预测值均较高.结论 RV-OT不同部位起源的VT有相应的心电图特征,其中I导联形态尤其具有定位价值,为RVOT心律失常起源提供了简便的定位标准.     

致心律失常右室发育不良心肌病的电生理基质和导管消融治疗(附二例报道)

目的报道2例致心律失常右室发育不良心肌病(ARVC)的电生理特征及导管消融结果。方法2例均以室性心动过速(简称室速)为首发症状。在窦性心律下,采用心脏电解剖标测系统构建右室并行起搏标测室速起源点。结果双极电压图上的低电压面积分别为36 cm2和48 cm2,室速起源于低电压的病变心肌与正常心肌的交界区,2例共有4种室速,采用线性消融后4种室速即刻均不能诱发,例2于术后第2天复发,但室率从188次/分降至160次/分,随访9个月和4个月未见室速复发。结论ARVC的电生理基质是病变心肌的低电压区,电解剖标测指导下的线性导管消融是一种有效的治疗方法。     

Arrhythmogenic right ventricular dysplasia presenting as right ventricular outflow tract tachycardia.

A case of a 51-year old male is presented. A left bundle branch block inferior axis tachycardia was manifest. At electrophysiological study this tachycardia was inducible and was ablated in the septal right ventricular outflow tract (RVOT). Two other tachycardias were identified both with right bundle branch block (RBBB) morphology raising the suspicion of diffuse pathology. Arrythmogenic right ventricular dysplasia (ARVD) was confirmed by right ventricular angiography and magnetic resonance imaging (MRI). An implantable cardioverter defibrillator (ICD) was implanted and an appropriate shock was later delivered.     

Spatial and transmural properties of the reentrant ventricular tachycardia circuit in arrhythmogenic right ventricular cardiomyopathy: Simultaneous epicardial and endocardial recordings

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Cryocatheter ablation of right ventricular outflow tract tachycardia

INTRODUCTION: Cryocatheter techniques have been successfully applied to treat supraventricular tachycardia but there are no reports on their value in treating ventricular tachycardia (VT). We present our initial experience with cryocatheter ablation of right ventricular outflow tract (RVOT) tachycardia. METHODS AND RESULTS: Cryocatheter ablation was attempted in 14 patients (13 females, age 45.9 +/- 12.7 years) who were highly symptomatic due to frequent monomorphic ventricular extrasystole (VES) or nonsustained VT originating within the RVOT. A 9-Fr, 8-mm-tip cryocatheter was used for both mapping and ablation. Cryoablation was started after localizing the arrhythmic focus by pace and activation mapping. Ablation success, defined by complete disappearance of target VES/VT acutely and during a follow-up of 9.3 +/- 1.4 weeks, was achieved in 13 of 14 patients. Ablation was successful with local activation times of 35 +/- 4 ms, 5.8 +/- 3.3 applications, 18.8 +/- 7.5 minutes total cryo time, 9.4 +/- 4.2 minutes fluoroscopy time, and 66.9 +/- 26.1 minutes total procedure time, the latter two measures showing a reduction with number of patients treated. Three patients reported slight pain related to local pressure of the catheter on the RVOT wall. No pain was described related to delivery of cryothermal energy. CONCLUSIONS: Initial experience shows that focal VES/VT originating in the RVOT can be successfully treated using cryocatheter ablation. Acute and short term success rates, fluoroscopy times, and duration of procedure are comparable to conventional ablation techniques. A major advantage seems to be the virtual absence of ablation related pain.     

Electrocardiographic determinants of the polymorphic QRS morphology in idiopathic right ventricular outflow tract tachycardia

Coupling Intervals and Polymorphic QRS Morphologies . Introduction: Premature ventricular contractions (PVCs) arising from the right ventricular outflow tract (RVOT) can trigger polymorphic ventricular tachycardia (PVT) or ventricular fibrillation (VF) in patients with no structural heart disease. We aimed to clarify the ECG determinants of the polymorphic QRS morphology in idiopathic RVOT PVT/VF. Methods and Results: The ECG parameters were compared between 18 patients with idiopathic PVT/VF (PVT‐group) and 21 with monomorphic VT arising from the RVOT (MVT‐group). The coupling interval (CI) of the first VT beat was comparable between the 2 groups. However, the prematurity index (PI) of the first VT beat was smaller in the PVT‐group than in the MVT‐group (P < 0.001). Furthermore, the QT index, defined as the ratio of the CI to the QT interval of the preceding sinus complex, was also smaller for the PVT/VF in the PVT‐group than that for the VT in the MVT‐group (P < 0.01). In the PVT‐group, the CI of the first VT beat was comparable between that of VT and isolated PVCs, but the PI of the first VT beat was shorter for VT than isolated PVCs (P < 0.05). The PI was the only independent determinant of the polymorphic QRS morphology (odd ratio = 2.198; 95% confidence interval = 1.321–3.659; P = 0.002). Conclusion: The smaller PIs of the first VT beat may result in a polymorphic QRS morphology. (Cardiovasc Electrophysiol, Vol. 23, pp. 521‐526, May 2012)     

Risk stratification of arrhythmogenic right ventricular cardiomyopathy based on signal averaged electrocardiograms

Background

Signal averaged electrocardiogram (SAECG) is a specific and non-invasive tool useful for arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis. However, its role in risk stratification of patients with ARVC remains largely undefined.

Methods

Sixty-four patients fulfilling Task Force ARVC criteria (mean age: 47 ± 14 years-old, 56% male, 50% definite ARVC) were enrolled. The baseline demographic, electrocardiographic, structural, and electrophysiological characteristics were collected. Patients with SAECG fulfilling all 3 Task Force criteria (3 + SAECG) were categorized into group 1, and those fulfilled 2 or less criterion were categorized into group 2. The study endpoints were unstable ventricular arrhythmia (VA), device detectable sustained fast VA (cycle lengths < 240 ms) and cardiovascular death.

Results

During a mean follow-up of 21 ± 20 months, 15 primary endpoints including 12 unstable VAs and 3 device-detected fast VAs were met. One patient died of electrical storm, and one patient underwent heart transplantation. The presence of 3 + SAECG predicted malignant events in all patients with definite and non-definite ARVC (p < 0.01, OR = 30.5, 95% CI = 2.5–373.7) and in patients with definite ARVC alone (p = 0.03, OR = 11.1, 95% CI = 1.3–93.9). Patients diagnosed with non-definite ARVC without 3 + SAECG were free from malignant events.

Conclusions

SAECG fulfilling all 3 Task Force criteria was an independent risk predictor of malignant events in ARVC patients. SAECG may play a valuable role in ARVC risk stratification.