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ARVC单形性室速: 导管消融还是ICD?,南京医科大学第一附属医院 邹建刚,5th VAS-CHINA,ARVC:并不罕见的心肌病,ARVC诊断标准2010,1. 心脏整体和/或局部运动障碍和结构改变 2.室壁病理组织学特征 3.复极障碍 4.除极或传导异常 5.心律失常 6.家族史 Circulation. 2010;121:1533-1541,ARVC室速,ARVC室性心律失常,主要条件 持续性或非持续性左束支传导阻滞型室性心动过速, 伴电轴向上 ( II、III、aVF QRS 负向或不确定, aVL 正向) 次要条件 持续性或非持续性右室流出道型室性心动过速, LBBB 型室性心动过速, 伴电轴向下( II、III、aVF QRS 正向或不确定, aVL 负向), 或电轴不明确 Holter显示室性早搏24 h 500个,ARVC:ICD植入指证 -ARVC-SCD的一级、二级预防,ICD therapy is indicated in patients with structural heart disease and spontaneous sustained VT, whether hemodynamically stable or unstable. ICD implantation is reasonable for the prevention of SCD in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) who have 1 or more risk factors for SCD.,(Class ,Level of Evidence: B),(Class a,Level of Evidence: C),IIa,ACC/AHA/HRS 2008guidelines for device-based therapy of cardiac rhythm 2012年指南关于ARVC猝死二级预防未作调整,指南关于ARVC猝死的一级预防,SCD危险因素: 有1个以上者植入ICD 作为SCD的一级预防 电生理检查诱发室性心动过速( VT) 心电监护的非持续性VT 男性 严重右室扩大, 广泛右室受累 发病很早( 5 岁) 累及左室 心脏骤停史 不能解释的晕厥,ARVC-VT/SCD:ICD植入的循证证据,BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition associated with the risk of sudden death (SD). METHODS AND RESULTS: We conducted a multicenter study of the impact of the implantable cardioverter-defibrillator (ICD) for prevention of SD in 132 patients (93 males and 39 females, age 40+/-15 years) with ARVC/D. Implant indications were a history of cardiac arrest in 13 patients (10%), sustained ventricular tachycardia in 82 (62%), syncope in 21 (16%), and other in 16 (12%). During a mean follow-up of 39+/-25 months, 64 patients(48%) had appropriate ICD interventions, 21 (16%) had inappropriate interventions, and 19 (14%) had ICD-related complications. Fifty-three (83%) of the 64 patients with appropriate interventions received antiarrhythmic drug therapy at the time of first ICD discharge. Programmed ventricularstimulation was of limited value in identifying patients at risk of tachyarrhythmias during the follow-up (positive predictive value 49%, negative predictive value 54%). Four patients (3%) died, and 32 (24%) experienced ventricular fibrillation/flutter that in all likelihood would have been fatal in the absence of the device. At 36 months, the actual patient survival rate was 96% compared with the ventricular fibrillation/flutter-free survival rate of 72% (P0.001). Patients who received implants because of ventricular tachycardia without hemodynamic compromise had a significantly lower incidence of ventricular fibrillation/flutter (log rank=0.01). History of cardiac arrest or ventricular tachycardia with hemodynamic compromise, younger age, and left ventricular involvement were independent predictors of ventricular fibrillation/flutter. CONCLUSIONS: In patients with ARVC/D, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias.Patients who were prone to ventricular fibrillation/flutter could be identified on the basis of clinical presentation, irrespective of programmed ventricularstimulation outcome.,Circulation. 2003 Dec 23;108(25):3084-91,ICD Therapy for prevention of SCD in ARVC Patients,132 pts (93 m, age 40+/-15 y) with ARVC ICD indications:history of cardiac arrest in 13 patients (10%) sustained VT in 82 (62%) syncope in 21 (16%), and other in 16 (12%) FU:39+/-25 m: 64 patients(48%) :appropriate ICD R 21 (16%) :inappropriate R 4 (3%) died At 36 months, the actual patient survival rate was 96% the ventricular fibrillation/flutter-free survival rate of 72% In patients with ARVC/D, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular,Circulation. 2003 Dec 23;108(25):3084-91,84 pts ARVC : ICD for SCD一级预防 FU: 4.7+/3.4y: 48% ICD intervention 19%:VF 5年生存率:伴1、2、3、4危险因子的为100%、83%、21%、15% EP诱发VT/VF、NSVT是独立预测因子,首次放电时间和放电次数,ICD电治疗的影响因子,危险因子对生存率的影响,结论: ARVC患者植入ICD作为SCD一级预防措施:接近一半患者可有效预防SCD,ARVC室速:导管消融,需要考虑的几个问题 ARVC室速的机制:疤痕折返,局灶 导管消融的成功率 远期复发率,J Am Coll Cardiol 2007;50:43240,24例患者 48次消融 随访3236months (range 1 day to 12 years),10次为三维电解剖标测,38次为常规方法标测 术后室速复发率高达85%,随访14个月无发作的比例仅为15%,且不同的标测方法之间未见显著性差异,即使术中消除所有诱发出来的室速,仍然有极高的复发率,南京医科大学心脏科 动态基质标测指导ARVC-VT消融,病例1,病例2,病例3,APEX,心动过速的标测,病例1:诱发一种类型室速,最早激动点和出口靠近基质边缘,无完整折返环,无舒张中期电位,无峡部。 病例2:有2种类型室速,其中一例有完整的折返环路和舒张中期电位,两种室速形态不同、激动传导方向相反,但有共同的传导通道位于三尖瓣环与基质边缘;一种室速的出口位于基质边缘,另一种室速出口远离基质。 病例3:诱发两种不同形态室速,无舒张中期电位,亦无峡部存在;一种室速起源于基质内并通过基质传导,出口位于基质边缘,另一种室速起源稍远离基质边缘,而出口远离基质。,VT1,VT2,病例2,病例3,12 Lead ECG (slower VT),Pacing at site A,Pacing at site B,结果,病例1、2的三种临床室速消融全部成功,但病例2仍可诱发一种新的非临床类型室速,室速频率快,电转复后未再行标测,后选用可达龙治疗。 病例3在完成两条线性消融后诱发出一种频率较慢的室速,经非接触球囊标测此慢频率室速通过两条消融线之间的间隙传导,消融此间隙后室速不再诱发。 平均放电次数17次,每条消融线达到双向传导阻滞。无手术并发症。平均随访20月,无心动过速发生。,ARVC-VT:心外膜消融,Percutaneous epicardial ablation of ventricular tachycardia after failure of endocardial approach in the pediatric population with arrhythmogenic right ventricular dysplasia 17例患者(14+/-4y),心内膜消融失败 20 VTs 诱发(2个大折返,18个局灶) 16例(94.1%)即刻成功 随访 26 15 (range 6 to 42)月 12人(70.6%)无室速发作,Heart Rhythm. 2010 Oct;7(10):1406-10,ARVC-VT:心外膜消融,Epicardial substrate and outcome with epicardial ablation of ventricular tachycardia in arrhythmogenic rightventricular cardiomyopathy/dysplasia. 33例患者中13例(39.4%)心内膜不能完全成功,需要行心外膜消融 13例心外膜消融后随访18+/-13 月 10/13(77%)无VT发作,Garcia FC, Circulation. 2009 Aug 4;120(5):366-75,ARVC-VT:消融的长期疗效,Outcomes of catheter ablation of ventricular tachycardia in arrhythmogenic right ventriculardysplasia/cardiomyopathy 87例患者,175次消融 平均随访88.366 月 1年,5年,10年无室速发作比例分别为47%,21%,15% 心外膜消融后1年,5年无室速发作比例64%,45%,Circ Arrhythm Electrophysiol. 2012 Jun 1;5(3):499-505,ARVC-VT消融:心内或和心外仍有较高复发率,但能显著减少VT负荷,In reported series of RV scar-related VT, abolition of inducible VT is achieved in 41%88% of patients During average follow-ups of 1124 months, VT recurs in 11%83% of patients, with some series observing a significant incidence of late recurrences increasing with time,Catheter ablation in ARVC/D can reduce frequent episodes of VT but long-term follow-up has demonstrated a continued risk of recurrence. Recommendations for ablation are as stated for ablation for VT associated with structural heart disease in the Indications section above,ARVC-VT:消融的现状与再认识,即刻成功率高 远期复发率也较高 三维标测结合心外膜消融明显提高成功率 即使完全消融成功,考虑VT复发,仍不能动摇ICD作为二级预防的适应证,Most patients who have VT related to structural heart disease will continue to have a standard indication for ICD therapy for primary prevention. Even when all VTs have been rendered non-inducible by ablation, the recurrence rate remains substantial so that secondary prophylaxis remains indicated.,ARVC-VT:消融的时机?,植入ICD之后? 植入后VT反复发作,药物效果欠佳, ATP成功率低,反复shock 但费用? 植入ICD之前? 预防性消融 减少发作,提高生活质量 如不植入ICD,有较大风险,病例:男性,33岁,ARVC+SMVT 2010年3月15日植入ICD,DFT测试:首次18J,失败;第二次,22J成功,植入时的参数设置,倍他乐克、可达龙,植入后3周:Electric storm,问题? 哪些患者需要早期,或先行消融后植入ICD,或ICD植入后尽早消融?,术前室速发作对AADs不敏感,药物不能终止或减少发作,预计植入后仍有较高的发生率 术中发现高DFT或术后住院期间观察到ATP效果欠佳 电风暴高危,ARVC植入ICD后电治疗的高危因素,History of cardiac arrest Ventricular tachycardia with hemodynamic compromise Younger age Left ventricular involvement Independent predictors of VF/ V Flutter 这些人是否应当早期行导管消融?,Circulation. 2003 Dec 23;108(25):3084-91,导管消融治疗ICD电风暴,Catheter ablation for the treatment of electrical storm in patients with implantable cardioverter-defibrillators: short- and long-term outcomes in a prospective single-center study. 95 pts (13 ARVC, 72 CAD, 10 DCM) 85 pts (89%) succeeded after 1-3 procedures FU:22 (1-43)m: 92% no ES,66% no VT; 11(12%) died,Circulation. 2008 Jan 29;117(4):462-9.,消融可有效治疗急性期ES,联合AAD可发挥长期保护作用,P

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