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ARVC單形性室速: 導(dǎo)管消融還是ICD?,南京醫(yī)科大學(xué)第一附屬醫(yī)院 鄒建剛,5th VAS-CHINA,ARVC:并不罕見的心肌病,ARVC診斷標(biāo)準(zhǔn)2010,1. 心臟整體和/或局部運(yùn)動障礙和結(jié)構(gòu)改變 2.室壁病理組織學(xué)特征 3.復(fù)極障礙 4.除極或傳導(dǎo)異常 5.心律失常 6.家族史 Circulation. 2010;121:1533-1541,ARVC室速,ARVC室性心律失常,主要條件 持續(xù)性或非持續(xù)性左束支傳導(dǎo)阻滯型室性心動過速, 伴電軸向上 ( II、III、aVF QRS 負(fù)向或不確定, aVL 正向) 次要條件 持續(xù)性或非持續(xù)性右室流出道型室性心動過速, LBBB 型室性心動過速, 伴電軸向下( II、III、aVF QRS 正向或不確定, aVL 負(fù)向), 或電軸不明確 Holter顯示室性早搏24 h 500個,ARVC:ICD植入指證 -ARVC-SCD的一級、二級預(yù)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年指南關(guān)于ARVC猝死二級預(yù)防未作調(diào)整,指南關(guān)于ARVC猝死的一級預(yù)防,SCD危險因素: 有1個以上者植入ICD 作為SCD的一級預(yù)防 電生理檢查誘發(fā)室性心動過速( VT) 心電監(jiān)護(hù)的非持續(xù)性VT 男性 嚴(yán)重右室擴(kuò)大, 廣泛右室受累 發(fā)病很早( 5 歲) 累及左室 心臟驟停史 不能解釋的暈厥,ARVC-VT/SCD:ICD植入的循證證據(jù),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一級預(yù)防 FU: 4.7+/3.4y: 48% ICD intervention 19%:VF 5年生存率:伴1、2、3、4危險因子的為100%、83%、21%、15% EP誘發(fā)VT/VF、NSVT是獨立預(yù)測因子,首次放電時間和放電次數(shù),ICD電治療的影響因子,危險因子對生存率的影響,結(jié)論: ARVC患者植入ICD作為SCD一級預(yù)防措施:接近一半患者可有效預(yù)防SCD,ARVC室速:導(dǎo)管消融,需要考慮的幾個問題 ARVC室速的機(jī)制:疤痕折返,局灶 導(dǎo)管消融的成功率 遠(yuǎn)期復(fù)發(fā)率,J Am Coll Cardiol 2007;50:43240,24例患者 48次消融 隨訪3236months (range 1 day to 12 years),10次為三維電解剖標(biāo)測,38次為常規(guī)方法標(biāo)測 術(shù)后室速復(fù)發(fā)率高達(dá)85%,隨訪14個月無發(fā)作的比例僅為15%,且不同的標(biāo)測方法之間未見顯著性差異,即使術(shù)中消除所有誘發(fā)出來的室速,仍然有極高的復(fù)發(fā)率,南京醫(yī)科大學(xué)心臟科 動態(tài)基質(zhì)標(biāo)測指導(dǎo)ARVC-VT消融,病例1,病例2,病例3,APEX,心動過速的標(biāo)測,病例1:誘發(fā)一種類型室速,最早激動點和出口靠近基質(zhì)邊緣,無完整折返環(huán),無舒張中期電位,無峽部。 病例2:有2種類型室速,其中一例有完整的折返環(huán)路和舒張中期電位,兩種室速形態(tài)不同、激動傳導(dǎo)方向相反,但有共同的傳導(dǎo)通道位于三尖瓣環(huán)與基質(zhì)邊緣;一種室速的出口位于基質(zhì)邊緣,另一種室速出口遠(yuǎn)離基質(zhì)。 病例3:誘發(fā)兩種不同形態(tài)室速,無舒張中期電位,亦無峽部存在;一種室速起源于基質(zhì)內(nèi)并通過基質(zhì)傳導(dǎo),出口位于基質(zhì)邊緣,另一種室速起源稍遠(yuǎn)離基質(zhì)邊緣,而出口遠(yuǎn)離基質(zhì)。,VT1,VT2,病例2,病例3,12 Lead ECG (slower VT),Pacing at site A,Pacing at site B,結(jié)果,病例1、2的三種臨床室速消融全部成功,但病例2仍可誘發(fā)一種新的非臨床類型室速,室速頻率快,電轉(zhuǎn)復(fù)后未再行標(biāo)測,后選用可達(dá)龍治療。 病例3在完成兩條線性消融后誘發(fā)出一種頻率較慢的室速,經(jīng)非接觸球囊標(biāo)測此慢頻率室速通過兩條消融線之間的間隙傳導(dǎo),消融此間隙后室速不再誘發(fā)。 平均放電次數(shù)17次,每條消融線達(dá)到雙向傳導(dǎo)阻滯。無手術(shù)并發(fā)癥。平均隨訪20月,無心動過速發(fā)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),心內(nèi)膜消融失敗 20 VTs 誘發(fā)(2個大折返,18個局灶) 16例(94.1%)即刻成功 隨訪 26 15 (range 6 to 42)月 12人(70.6%)無室速發(fā)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%)心內(nèi)膜不能完全成功,需要行心外膜消融 13例心外膜消融后隨訪18+/-13 月 10/13(77%)無VT發(fā)作,Garcia FC, Circulation. 2009 Aug 4;120(5):366-75,ARVC-VT:消融的長期療效,Outcomes of catheter ablation of ventricular tachycardia in arrhythmogenic right ventriculardysplasia/cardiomyopathy 87例患者,175次消融 平均隨訪88.366 月 1年,5年,10年無室速發(fā)作比例分別為47%,21%,15% 心外膜消融后1年,5年無室速發(fā)作比例64%,45%,Circ Arrhythm Electrophysiol. 2012 Jun 1;5(3):499-505,ARVC-VT消融:心內(nèi)或和心外仍有較高復(fù)發(fā)率,但能顯著減少VT負(fù)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:消融的現(xiàn)狀與再認(rèn)識,即刻成功率高 遠(yuǎn)期復(fù)發(fā)率也較高 三維標(biāo)測結(jié)合心外膜消融明顯提高成功率 即使完全消融成功,考慮VT復(fù)發(fā),仍不能動搖ICD作為二級預(yù)防的適應(yīng)證,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:消融的時機(jī)?,植入ICD之后? 植入后VT反復(fù)發(fā)作,藥物效果欠佳, ATP成功率低,反復(fù)shock 但費(fèi)用? 植入ICD之前? 預(yù)防性消融 減少發(fā)作,提高生活質(zhì)量 如不植入ICD,有較大風(fēng)險,病例:男性,33歲,ARVC+SMVT 2010年3月15日植入ICD,DFT測試:首次18J,失??;第二次,22J成功,植入時的參數(shù)設(shè)置,倍他樂克、可達(dá)龍,植入后3周:Electric storm,問題? 哪些患者需要早期,或先行消融后植入ICD,或ICD植入后盡早消融?,術(shù)前室速發(fā)作對AADs不敏感,藥物不能終止或減少發(fā)作,預(yù)計植入后仍有較高的發(fā)生率 術(shù)中發(fā)現(xiàn)高DFT或術(shù)后住院期間觀察到ATP效果欠佳 電風(fēng)暴高危,ARVC植入ICD后電治療的高危因素,History of cardiac arrest Ventricular tachycardia with hemodynamic compromise Younger age Left ventricular involvement Independent predictors of VF/ V Flutter 這些人是否應(yīng)當(dāng)早期行導(dǎo)管消融?,Circulation. 2003 Dec 23;108(25):3084-91,導(dǎo)管消融治療ICD電風(fēng)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.,消融可有效治療急性期ES,聯(lián)合AAD可發(fā)揮長期保護(hù)作用,P
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