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1、Tako-tsubo综合征,南京市第一医院心内科 南京市心血管病医院 田乃亮,一、概述,三国演义里的周瑜“赔了夫人又折兵”后大病不起 1990年日本的Hikaru Sato教授，心尖球形综合征（Apical Ballooning Syndrome) 心室造影特征：收缩末期左心室造影为圆形底部和狭窄的颈部 日本，欧洲，美国报道多,一、概述,心室造影显示，左心室形状类似烧瓶圆底和窄的瓶颈（round bottom and narrow neck）， 形状很像日本用来捕捉章鱼的瓶子。因此，Sato 教授将之命名为“Tako-tsubo” 心肌病。日文 Tako是章鱼（Octopus），tsubo是瓶
2、子,其它名称,急性左心室球形改变（acute left ventricular ballooning），可逆性应激性心肌病（reversibe stress cardiomyopathy），破碎心脏综合征（broken heart syndrome）和应激诱发的心肌顿抑（stress induced stunning）,一、概述,特点 突然的类似心绞痛样胸痛 ECG：典型的ST段抬高、多导联T波倒置和异常的QS波 UCG or Left ventriculogram:前壁下部和心尖部非连续的室壁运动异常 心肌酶：轻度升高 临床表现类似MI，但CAG无明显血流动力学狭窄证据,一、概述,可逆性心室
3、收缩功能损害 突然起病，快速好转 女性多见：Monica系列研究，女性88.8%,年龄10-89岁，平均58-77岁 日本 女/男 为 7/1,女性68.612.2岁,男性65.99.1岁(Circulation,2000),ECG,二、流行病学,发病率？美国Bybee报道，2002-2003年STEACS 2.2%心尖球形综合征。Ito可疑ACS中，心尖球形综合征1.7%,Matsuka报道2.2% 。入院时表现为AMI的突发心衰,异常Q波和ST-T改变中,Akashi报道2.0%,二、流行病学,冠心病危险因素评价 高血压43%，糖尿病11%，血脂异常25%，吸烟23%,三、主要症状,诱因：
4、心理或生理应激 26.8%患者有亲友意外死亡、家庭虐待、争吵、灾难性医学诊断、生意亏损 37.8%过度劳累、哮喘发作、胃镜检查、全身疾病恶化 50%有明确诱因,三、主要症状,类似心绞痛样胸痛和呼吸困难、晕厥 胸痛为主占67.8%,呼吸困难17.8% 心源性休克4.2%,急性肺水肿 室性心律失常,室速、室颤1.5%， 左心室血栓形成可导致TIA、脑梗死、肾梗死，偶左室破裂,四、辅助检查,ECG ST抬高（90%），存在数小时，和T波倒置（97%），心前导联ST抬高83.9%, T波异常64.3%,Q波31.8%,QT间期延长 心肌损伤标记物 肌钙蛋白阳性86.2%(入院48H内),CK-MB升高
5、73.9%,注意：轻微升高,与受累心肌节段不平行,四、辅助检查,CAG 无典型的阻塞性病变，正常或50%的狭窄 左心室造影:左心功能不全，EF平均20%-49%，但在7-30天快速改善， EF平均上升60%-76% 急性期，中到重度的心室中部及心尖运动不能或运动障碍，伴心底部功能正常或运动过度，恢复后运动异常消失,四、辅助检查,UCG：左室心尖部运动减低伴基底段收缩力增强 运动减弱范围超过单支冠脉供血区域 MRI:无心肌坏死的证据 神经体液因素测定:儿茶酚胺和神经肽,Wittstein等报道(NEJM),13例(共19例)患者住院1-2天血浆儿茶酚胺（尤其肾上腺素）水平是AMI2-3倍,是正常
6、人7-34倍,BNP与左室收缩功能变化一致 心内膜心肌活检:单核淋巴细胞和巨噬细胞浸润，心肌细胞收缩带的坏死,病毒抗体测定无心肌炎症,Mayo Clinic的Kevin Bybee教授诊断标准：,1.新发现的心电图异常ST段抬高或T波倒置 2.冠状动脉造影没有冠状动脉闭塞性病变 3.一过性可逆左心室不运动或者运动减弱 4.无心肌病、头颅创伤、脑出血或嗜铬细胞瘤,诊断,日本文献的标准 1、暂时性左室心尖部气球样变；2、无明显冠状动脉狭窄；3、无其它心脏病 美国采用如下的标准：1、心尖部气球样变伴有无动力学和动力学紊乱状态，超过了单支冠脉供血范围；2、住院24小时内冠脉造影没有50的狭窄；3、新出
7、现心电图ST-T异常。有些学者认为尚需排除陈旧性心肌梗塞，瓣膜性心脏病，蛛网膜下腔出血及嗜铬细胞瘤等,鉴别诊断,嗜铬细胞瘤，该病也可以出现一过性tako-tsubo样暂时性左室功能不全，应诊断为儿茶酚胺性心肌病 暴发性心肌炎(所有被评价的病人的病毒抗体均为阴性),鉴别诊断,ACS ：左室心尖部气球样变时，心肌坏死标志物仅轻度升高，冠状动脉无明显狭窄，心尖部室壁运动异常及心功能下降短期内完全恢复正常 Wittstein et al. found that women presenting with Takotsubo cardiomyopathy had significantly higher
8、 catecholamine levels than women presenting with classic acute myocardial infarction, despite experiencing similar episodes of emotional stress.,鉴别诊断,echocardiography revealed akinesis of the apex and the mid-ventricle as well as basal hyperkinesis, wall-motion abnormalities extending beyond the reg
9、ion supplied by one coronary artery. This feature is characteristic of Takotsubo cardiomyopathy, whereas wall-motion abnormalities observed in acute myocardial infarction are often more localized.,鉴别诊断,Ibanez et al. suggest that this cardiomyopathy might result from a transient LAD obstruction cause
10、d by a ruptured artherosclerotic plaque located proximally in a large LAD that extends to the diaphragmatic surface of the left ventricle.(IVUS of five patients). Early reperfusion follows, resulting in a widely stunned, rather than infarcted myocardium. Coronary vasospasm does not appear to play a
11、significant part in the disorder,特点总结,强烈的心理应激诱因 老年绝经后女性多见(60岁),M/F为1:6 胸痛、呼吸困难、晕厥 ECG：ST抬高、T倒置 UCG：短暂的左室心尖-中段气球样变，运动减低伴基底段收缩力增强,特点总结,心肌酶学升高不明显 CAG正常 心室造影：左室心尖-中段心腔扩大、基底段缩窄 预后良好、康复迅速(2-4周) 也可见于右室,五、预后,良好，住院死亡1.1% 心衰伴或不伴肺水肿发生率17.7%,复发率3.5%,六、发病机制,冠脉痉挛 微血管功能障碍 应激引起过度交感神经激活，儿茶酚胺水平明显高，引起神经源性心肌顿抑，导致细胞内钙超载
12、，氧自由基增加。雌激素缺乏增强这一反应 程度不同的地域性心肌炎，但无感染史，炎症指标无动态结果 儿茶酚胺介导心室基底段运动亢进，可促发左室流出道动力性梗阻，甚至心尖部运动减弱气球样变 家族史 SPECT：心肌灌注减少,提示冠脉微循环受损 目前观点：应激导致交感兴奋和血浆儿茶酚胺水平过渡升高，引起心肌运动障碍（WMA）,七、治疗,去除诱因 对症和支持性疗法，包括吸氧，使用吗啡。左心室流出道梗阻，需要阻断剂 利尿、扩血管药，ACEI或ARB， 阻断剂，避免-受体激动剂(多巴胺,多巴酚丁胺等) 患者的血液动力学失代偿和不稳定，可能需要使用升压药物和主动脉内球囊反搏泵 （IABP） 心室血栓需抗凝,目
13、前问题,漏诊多 医生仅满足CAG,很少行左室造影 急性期未行UCG检查，恢复期无随访 医生认识不足 急性事件发生与进行心导管术存在时间延搁,八、尚待研究的问题,中老年女性易发的原因？ 强烈应激反应触发该病的机制？ 为何左心室易发生？ 首先，从解剖上看，左心室心尖部缺乏其它部位心室壁所具有的三层心肌环绕的结构；其次，从血供角度看，心尖部血供属于冠状动脉的终末部分，当发生血液供应障碍时，容易首先受累，并且，在发生过度扩张后，心尖部为更容易失去弹性,A case of Takotsubo cardiomyopathy mimicking an acute coronary syndrome,A 71
14、-year-old woman acute, left-sided, substernal chest pain at rest. Her husband had died 4 months previously, causing her severe emotional stress, and she was also in the process of selling her home.,medical history,peripheral vascular disease, type 2 diabetes mellitus, hypertension, hypothyroidism, a
15、nd rheumatoid arthritis. The patient denied having any history of angina symptoms. She had undergone a nuclear stress test 2 weeks before presentation, but this had not revealed any evidence of ischemia.,presentation,T afebrile, BP 72/50 mm Hg, HR 72 次/min, R 18 次/min ,Sa2 99%. 2/6 systolic murmur a
16、t the apex of the heart, without radiation. No jugular venous distention or lower-extremity edema was noted, and the lungs were clear on auscultation.,The patients complete blood count, basic metabolic panel and liver-function tests were all within the normal range. Two sets of myocardial enzyme ass
17、ays showed an increase in creatine phosphokinase from 84 U/l to 121 U/l (normal range 24170 U/l), and in troponin I from 0.46 g/l to 1.26 g/l (normal range 00.05 g/l) over 2 h.,Metzl MD et al. (2006) A case of Takotsubo cardiomyopathy mimicking an acute coronary syndrome Nat Clin Pract Cardiovasc Me
18、d 3: 5356 doi:10.1038/ncpcardio0414,Figure 1 A 12-lead electrocardiogram showing ST-segment elevations and T-wave inversions in the right precordial leads, which is a typical pattern observed in Takotsubo cardiomyopathy,CAG,Cardiac catheterization revealed TIMI grade III flow in all coronary arterie
19、s and a 40% lesion in the proximal right coronary artery. The left anterior descending (LAD) artery wrapped around the apex of the heart,Metzl MD et al. (2006) A case of Takotsubo cardiomyopathy mimicking an acute coronary syndrome Nat Clin Pract Cardiovasc Med 3: 5356 doi:10.1038/ncpcardio0414,Figu
20、re 2 Left ventriculogram of the patient during systole showing mid, distal and apical left ventricular ballooning, with vigorous contraction of the basal segment as seen in Takotsubo cardiomyopathy,transthoracic echocardiogram,similar wall motion to that observed by ventriculogram, systolic anterior
21、 motion of the mitral valve leaflets, and a LVEF of 35% 3 days revealed improved left ventricular wall motion, no systolic anterior motion of the mitral valve, and a LVEF of 45%. Follow-up echocardiography at 6 weeks revealed normal left ventricular function and an ejection fraction of 55%.,Treatmen
22、t and management,underwent thrombolysis with tenecteplase and was given heparin via intravenous drip. aspirin, -blockers, angiotensin-converting-enzyme inhibitors, cardiac catheterization and intravenous diuretics if needed. statins,CASE 2,女，66岁 胸闷胸痛1小时 诱因未明确,ECG：前壁心肌缺血,UCG：左室心尖至中部运动降低，基底部运动增强,CAG：正常，左室造影类似UCG,治疗,阿司匹林，阿托伐他汀，倍他乐克，华法令 3月后复查UCG提示EF68%（入院EF50%）,谢谢！,