應(yīng)激性心肌病Stress-Induced Cardiomyopathy課件

1、Stress-Induced Cardiomyopathy(Tako-tsubo syndrome)應(yīng)激性心肌病 澳門 鏡湖醫(yī)院心內(nèi)科金 椿病情介紹女性，70歲 (住院號(hào)：08-4361) 主訴：胸痛1小時(shí)。AED (2008.2.28 21:45)： BP 156/84mmHg, HR 90bpm EKG 2008.2.28 21:542008.2.28 23:372008.2.29 08:13心肌酶譜變化 參考值日期CK(96-140U/L)CKMB(25U/L)TNT(50%), or spontaneous vasospasm in all patients Left ventricu

2、lography akinesia in the anterolateral, apical, diaphragmatic, septal areas as well as base hypercontractile The median EF of the LV was 30.4% . End-diastolic and end-systolic frames of the LV (A and B) and RV (C and D) demonstrating extent of LV and RV dysfunction (arrows). Echocardiogram Apical tw

3、o chamber echocardiographic view showing LV apical ballooning and sigmoid septum End-diastolic and end-systolic apical four-and-two chamber echocardiographic views demonstrating the typical apical and mid-ventricular LV wall-motion abnormalities of a patient with takotsubo cardiomyopathy 14 studies:

4、2% of ST elevation infarcts, most cases in post-menopausal women. chest pain and dyspnoea in 67.8 and 17.8% Cardiogenic shock (4.2%) ventricular fibrillation (1.5%)ST-segment elevation( 81.6%)T wave abnormalities( 64.3%)Q waves( 31.8%)Cardiac biomarkers mildly elevated( 86.2%)LV dysfunction on admis

5、sion EF 20 to 49%, over a period of days to weeks. preceded by emotional (26.8%) or physical stress (37.8%).Norepinephrine concentration was elevated ( 74.3% ) excellent, with full recovery in most patients. In-hospital mortality was 1.1%. Only 3.5% of the patients experienced a recurrence. Comparis

6、on between positron emission tomography (A, C, and E) and single-photon emission computed tomography (B, D, and F) images: metabolic image revealed severely reduced F-18 fluorodeoxyglucose uptake in the apical and mid-ventricular segments compared with perfusion abnormalities. (A and B) Horizontal l

7、ong-axis; (C and D) vertical long-axis; (E and F) short-axis. Light microscopy Endomyocardial biopsy specimen: contraction-band necrosis (arrows) and small amounts of mononuclear cell infiltration (haematoxylin and eosin stain). (A) Original magnification x100; (B) original magnification x200. PAS s

8、taining (arrows) shows remarkable intracellular accumulation of glycogen (A). After functional recovery only small amounts of glycogen particularly around the nuclei of myocytes (arrows) were documented (B). Electron microscopy Electron microscopy of acute biopsies showing numerous vacuoles of diffe

9、rent sizes and contents (myelin bodies, residual cellular products), loss of contractile material, and areas of non-specified cytoplasm (A). The interstitial space was widened containing formation of cellular debris (B). In the acute phase, formation of myelin bodies could be documented (C). In TTC

10、contraction bands of sarcomeres were found (D). Recovered biopsies showed a nearly complete rearrangement of contractile material with regularly distributed sarcomeres, normal nuclei, and mitochondria (E, F). vac, vacuole; svac, small vacuoles; N, nucleus; cyt, cytoplasm; mit, mitochondria; cd, cell

11、ular debris; mb, myelies bodies; sarc, sarcomeres; cb, contraction band. Immunohistochemistry Immunohistochemistry of intracellular proteins (specific labelling green, phalloidin red, nuclei blue). -actinin was detected only in the border zone during TTC (A). After functional recovery a regular dist

12、ribution was found (B). N-terminal dystrophin showed a decrease in TTC verifying a loss of protein-to-protein interaction (C) in comparison with biopsies after functional recovery (D). C-terminal dystrophin was unaltered in TTC suggesting that integrity of the sarcolemma is maintained (E, F). Connex

13、in-43 showed a reduced cellcell connection in TTC (G), whereas a myocardial integrity was documented after functional recovery (H). Immunolabelling for titin was performed using T12 (A, B) and Tz1/Z2 (C, D). F-actin (red) was visualized with TRITCconjugated phalloidin and nuclei (blue) were counters

14、tained with Draq-5. Note that titin in the acute stage (A, C) is either absent in the central parts of the myocytes or shows a punctuated pattern as compared with a clear cross-striated pattern of labelling and higher expression levels in the recovery phase (B, D). Immunohistochemistry of extracellu

15、lar proteins (specific labelling green, phalloidin red, nuclei blue). The ECM stained by fibronectin (A, B) and collagen-1 (C, D) was increased and the myocardial syncytium was separated. After functional recovery, a decrease of extracellular proteins was observed. Macrophages (arrows) showing infla

16、mmatory response were regionally accumulated in TTC (E, F). Slight increase of T-lymphocytes (arrows) was regionally observed in TTC (G, H). Pathophysiology precise mechanisms are unknown catecholamine-mediated mechanisms with likely mediation via cardiac sympathetic nerves.Sudden surging catecholam

17、ine levels, can be precipitated by emotional or physical stress Catecholamine levels are characteristically far higher than in matched patients catecholamine-mediated multivessel epicardial spasm, microvascular coronary spasm, or possible direct catecholamine-mediated myocyte injury. Pathophysiology

18、 Pathophysiology On myocardial biopsy, the histological appearances are very similar to contraction band necrosis seen in phaeochromocytoma In a rodent model, TTC can be prevented with - or -blockade The more dense distribution of adrenoceptors at the apex might explain why the apex is affected whil

19、e the base is spared In addition, oestrogen downregulates cardiac adrenoceptors and attenuates their response to activation, providing a plausible reason why the condition is largely confined to postmenopausal women Mayo Clinic criteria for tako-tsubo cardiomyopathy 1,Transient,reversible akinesis or dyskinesis of the left ventricular apical and mid-ventricular segments with regional wall motion