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1、肥厚性心肌病的器械治疗,阜外心血管病医院 滕思勇,内容提要,HCM的基本特征 HCM的ICD 治疗进展 HCM的DDD治疗进展,肥厚性心肌病（HCM）是一种复杂的、相对常见的遗传性心脏疾病。 经过40年的严密调查和注册研究，发现 HCM是所有年龄段的患者致残和死亡的重要原因。 由于临床表现、自然史和预后的显著差异，对于心血管专家来说，HCM的治疗依然存在许多争议。,基本特征（1）,HCM年死亡率约为1.4%，其中猝死0.7%，心衰0.5%，中风0.2%。 猝死可为HCM的首发表现。猝死也可发生在疾病平稳期 虽然大部分猝死发生于青少年，但猝死并不局限于青少年，猝死会持续在所有年龄组中发生,基本特
2、征（2）,猝死的主要危险因素: 持续室速 家族性猝死史 恶性突变类型（如：-MHC基因Arg403-Gln的家系） 晕厥史 反复发作的非持续性室速 左室肥厚（室壁30mm）,基本特征（3）,HCM的基本特征 HCM的ICD 治疗进展 HCM的DDD治疗进展,内容提要,心脏性猝死是肥厚性心肌病患者死亡的常见原因。大约有10的肥厚性心肌病患者被认为有心脏性猝死的危险性。 肥厚性心肌病猝死高危患者：( 1)猝死幸存者；(2)自发持续性心动过速；( 3)猝死家族史；( 4)不明原因晕厥史；(5)运动后血压反应异常，收缩压不升高反而降低者；(6)左室壁或室间隔厚度30mm；流出道压力阶差50mmHg。
3、50以上的肥厚性心肌病高危患者10年内将发生心脏性猝死。 肥厚性心肌病是35岁以下运动员心脏性猝死的最主要原因。,心脏性猝死的一级和二级预防的多个前瞻性多中心随机临床试验的结果（AVID、CASH、MADIT、MADIT-、MUSTT、SCD-HeFT、COMPANION）已经充分证明ICD是肯定的效果最佳和唯一可靠的预防心脏性猝死的选择，能够有效降低心脏性猝死高危患者的病死率。,2008年ACC /AHA /ESC室性心律失常治疗和心脏性猝死预防指南把SCD一级和二级预防的建议合并，对SCD的一级预防提到更加显著位置，HCM患者出现以下情况为植入ICD类适应症：1）自发持续性VT、无论血液动
4、力学是否稳定。2）有晕厥史、电生理检查明确诱发有血液动力学不稳定的持续性VT或VF。 肥厚型心肌病患者有一项以上主要SCD危险因素，包括心脏骤停史、自发持续性VT、自发非持续性VT、SCD家族史、不明原因晕厥史、左室厚度30mm、运动时血压反应异常，建议植入ICD。-,心脏性猝死（SCD）的发病年龄 Single most frequent cause of SCD in youn competitive athletes in the U.S ARVC, arrhythmogenic right ventricular cardiomyopathy; AS, aortic valve ste
5、nosis; CAD, coronary artery disease; CHD, *Regarded as possible (but not definitive) evidence for hypertrophic cardiomyopathy at autopsy with mildly increased LV wall thickness (1519 mm) and heart weight (44776 g). Includes most commonly, Kawasaki disease, sickle cell trait and sarcoid.,Maron BJ,Cir
6、culation 2009; 119: 1085 1092,HCM心律失常的发生具有不可预测性 Time interval between implantation of implantable cardioverter-defibrillator (ICD) and first appropriate intervention. Variable time delay after implantation is evident, with some device discharges occurring relatively early and others 510 years later
7、(blue bars). Hourly distribution of appropriate ICD interventions over the 24-h day for 126 ventricular tachycardia/ ventricular fibrillation events in 63 patients with HCM.,心脏猝死的危险分层 Pyramid profile currently used to identify patients at highest risk for SCD who are potential candidates for an impl
8、antable cardioverter-defibrillator (ICD). BP, blood pressure; LV, left ventricular; LVH, left ventricular hypertrophy;NSVT, nonsustained ventriculartachycardia; VT, ventricular tachycardia. *Following alcohol septal ablation, sustained VT has been reported in a significant minority of patients (10%)
9、 over the short term. Direct relation between magnitude of LV hypertrophy (maximummax wall thickness by echocardiography)and SCD risk. Mild hypertrophygenerally conveys lower riskand extreme hypertrophy (wall thickness30 mm) is associated with thehighest risk.,HCM合并持续性室性心动过速的影像和病理特征 (A)Massive hyper
10、trophy with ventricular septal (VS) thickness of 55 mm. (B) Akinetic thin-walled LV apical aneurysm with midcavity muscular apposition. D, distal (cavity); LA, left atrium; P, proximal (cavity); (B1) Contrast-cardiovascular magnetic resonance shows delayed enhancement (ie, scar) involving the thin a
11、neurysm rim (arrowheads) and contiguous myocardium (large arrow); small apical thrombus is evident (small arrow). (C) Large transmural ventricular septal scar (arrow) resulting from alcohol ablation(arrow) (reproduced with permission). (D) “End-stage” heart showing extensive and transmural septal sc
12、arring, extending into the anterior wall (arrowheads).,HCM心脏核磁显像延迟提示致心律失常基质的存在 Ventricular tachyarrhythmias on ambulatory (Holter) ECG, including nonsustained VT (NSVT), are significantly more frequent in the presence of DE. PVC, premature ventricular contraction; SVT, supraventricular tachycardia.
13、A 21-year-old man with hypertrophic cardiomyopathy (HCM) and septal scarring who survived an episode of ventricular fibrillation (VF) because of ICD intervention (A). Contrast-enhanced CMR image showing transmural DE with high signal intensity occupying substantial proportion of septum (arrows). (B)
14、 Without contrast, showing moderate asymmetric hypertrophy of the ventricular septum (VS; 21 mm). (C) Intracardiac electrogram showing VF interrupted by defibrillation shock (arrow).,Maron BJ, Am J Cardiol 2008;,肌节组成及突变示意图 Schematic representation of the components of a half sarcomere. Components in
15、 which cardiomyopathy-associated mutations are found are underlined.,MHC突变Arg403-Gln猝死患者的心肌细胞形态特征 A, Gross heart specimen from a 13-year-old male competitive athlete showing isproportionate thickening of the ventricular septum (VS) with respect to the left ventricular (LV) free wall (RV indicates ri
16、ght ventricular wall); B, marked disarray of cardiac muscle cells in the isproportionately thickened VS with adjacent hypertrophied cells arranged in a chaotic pattern at oblique and perpendicular angles, forming the typical disorganized architecture of HCM; C, LV myocardium showing several abnormal
17、 intramural coronary arteries with markedly thickened walls and narrowed lumen, dispersed within replacement fibrosis,Maron, B. J. JAMA 2002;287:1308-1320,Copyright restrictions may apply.,糖原储积性疾病表现为HCM和WPW,LAMP2型心肌病特征 (A) From a 14-year-old boy with sudden cardiac death and a septal thickness of 65
18、 mm (heart weight 1,425 g). (B) Clusters of myocytes with vacuolated sarcoplasm (stained red) embedded in an area of scar (stained blue; Masson trichrome). (C) Disorganized arrangement of myocytes most typical of sarcomeric hypertrophic cardiomyopathy.(D) Intracardiac electrogram. The implantable ca
19、rdioverter-defibrillator elicited 5 defibrillation shocks that failed to interrupt ventricular fibrillation (280 beats/min).,心脏性猝死的家族史 (Top) Intracardiac electrogram obtained at 1:20 am while asleep 5 years after placement of an implantable cardioverter-defibrillator (ICD). From a 35-year-old man wi
20、th hypertrophic cardiomyopathy who received prophylactic ICD because of family history of SCD and marked ventricular septal thickness (31 mm). (A) Ventricular tachycardia (VT) begins abruptly, at 200 beats/min. (B) Defibrillator senses VT and charges. (C) VT deteriorates into ventricular fibrillatio
21、n (VF) (D) defibrillator issues 20-J shock (arrow), restoring sinus rhythm immediately. Virtually identical sequence occurred 9 years later during sleep; patient is now 52 years old and asymptomatic. (Reproduced with permission.) (Bottom) Flow-chart of ICD-related outcome in 506 highrisk HCM patient
22、s from an international, multicenter ICD registry,HCM主要危险因素与预后 (Top) Appropriate implantable cardioverter-defibrillator (ICD) intervention rates (per 100 person-years) are not significantly different with respect to 1, 2 or 3 risk factors. (Bottom) Cumulative rates for first appropriate device inter
23、vention in patients with 1, 2 or 3 risk factors.,Maron BJ,Circulation 2009; 119: 1085 1092,HCM的基本特征 HCM的ICD 治疗进展 HCM的DDD治疗进展,内容提要,肥厚型心肌病伴窦房结功能异常或房室传导阻滞需植入永久性起搏器者。（伴或不伴左室流出道梗阻） 药物治疗无效、静息或应激时流出道梗阻的肥厚型心肌病患者，不推荐植入永久性起搏器，为IIb类（证据级别A级）推荐。（强调个体化治疗） 有SCD风险（主要SCD风险：心脏骤停史，自发持续性VT，自发非持续性VT，SCD家族史，晕厥，左室厚度30mm，运
24、动时血压反应异常；可能的SCD风险：房颤、心肌缺血、左室流出道梗阻、突变高危、强竞技性体力活动时）应植入DDD-ICD。 无症状或有症状但药物可控制、没有左室流出道梗阻证据的肥厚型心肌病患者禁止植入永久性起搏器。,HCM起搏器治疗指南（2008年AHA/HRS),HCM梗阻型的血流动力学异常,LV流出道,二尖瓣前叶,增厚的间隔,舒张期,收缩期,1101例HCM随访6.36.2年。结论：休息下LVOT压差30mmHg是HOCM死亡、心衰的独立危险因素。,Martin S N Engl J Med 2003; 348:295-303,DDD起搏器植入后,舒张期,收缩期,起搏 导线,经导管测流出道压
25、差,起搏前,起搏治疗后,LV 收缩压,动脉压,DDD起搏器植入后,多中心,随机,双盲,3个月交叉试验 药物治疗无效的83例患者，平均年龄53岁,随访1年。比较主动起搏（DDD，最适AV间期）对非主动起搏（AAI起搏，30次/分）的疗效。 84%患者生活质量和症状改善.,PIC 研究,药物治疗无效的48例患者,LVOT压差50mmHg 随机双盲3个月交叉试验 在双盲试验阶段，患者生活质量和症状改善,DDD起搏和AAI起搏组无差别。 6个月非盲试验阶段 患者生活质量和症状在DDD组明显改善。 安慰剂效应？,M-PATHY 研究,DDD起搏治疗梗阻型HCM的适应症,LVOT压差静息30mmHg 激发
26、后50mmHg 室间隔基部而非心尖部、心室中部的心肌肥厚 无慢性或频繁发作的阵发性房颤,DDD起搏治疗梗阻性HCM的技术参数,心室起搏位点：起搏电极必须置于真正的右室尖 AV间期（25msec125msec)：必须短于窦性心律的PR间期 AV间期程控期间监测左室和主动脉压力，必要时行激发试验 尽量保证窦性心律,最佳AV间期的程控,最佳AVD调整原则： 有效的AVD短于自身PR间期 保持适当房室同步，维持左室的前负荷 Jeanrenaud观察13例不同AVD的血流动力学变化,Lancet,1992,339:1318-1323,DDD起搏治疗不能改善LVOT梗阻的原因,起搏器参数程控不当 心室激动
27、提前不适当（AV延迟值过长） 干扰左房排空（ AV延迟值过短） 其他相关的异常 心室电极位于室间隔的近端或高位 异位乳头肌阻塞LOVT 原发的二尖瓣反流 左室腔中部梗阻 心房或/和心室的快速心律失常 左室舒张功能障碍 药物治疗不当,DDD起搏对HOCM的远期疗效仍争论，没有证据表明DDD起搏可改善HOCM患者的预后。 严格选择病例-HOCM伴窦性心动过缓/不能耐受BB者 程控最佳的AVD 药物控制房颤,争论还将继续？,Simultaneous measurement of aortic and left ventricular pressures.,Haruki S et al. Eur J
28、Heart Fail 2010;12:94-97,Published on behalf of the European Society of Cardiology. All rights reserved. The Author 2009. For permissions please email: journals.permissionsoxfordjournals.org.,Simultaneous measurement of aortic and left ventricular pressures. (A) The baseline pressure gradient was 12
29、7 mmHg. (B) DDD pacing at a rate of 80/min and an atrioventricular interval of 150 ms reduced the gradient to 27 mmHg. (C) After 5 min of disopyramide infusion (1.0 mg/kg) without DDD pacing, the gradient was 28 mmHg. (D) The combination of DDD pacing with an atrioventricular interval of 150 ms and disopyramide reduced the gradient to 4 mmHg.,Thank You !,,