肥厚性心肌病的器械治疗.ppt
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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
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- 肥厚 心肌 器械 治疗
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