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1.
心脏移植已经成为终末期心力衰竭的最终有效治疗方式。但术后移植心脏血管病变严重影响患者长期存活。鉴于移植心脏去神经化导致的无症状或症状极不明显的特征,亟需一项有效、无创、适合国人的移植心脏血管病变诊断筛查工具。冠状动脉计算机断层扫描由于其较高的冠状动脉病变诊断价值成为一项比较令人满意的移植心脏血管病变诊断筛查工具,可以在国内尚未建立起冠状动脉造影或血管内超声筛查诊断移植心脏血管病变系统的情况下,发挥巨大的临床和预防作用,有利于患者自身利益和国内心脏移植的发展。  相似文献   

2.
目的观察单纯心壁病损造成心脏扩张者心脏的病理形态特征,为临床和影像提供鉴别诊断参考依据。方法2004年6月至2006年6月底,对阜外心血管病医院进行心脏移植的56例受体心脏在离体后立即进行了肉眼观察、测量和摄影记录,并进行了全面的病理组织学观察。以其中单纯心壁病损造成的心脏扩张者进行临床病理对比分析,观察其形态特点。结果56例受体心脏中38例为单纯心壁病损造成的心脏扩张者,占67.9%。这38例中50.0%为原发性扩张型心肌病,23.7%为致心律失常性右心室心肌病,15.8%为缺血性心肌病,其余的10.5%为局灶性心肌致密化不全、巨细胞性心肌炎和特异性心肌病中的酒精性心肌病和高血压性心肌病。38例中临床诊断与病理诊断不相同的有15例,不相符率为39.5%,不相符率较高的依次为巨细胞性心肌炎(100%)、缺血性心肌病(83.3%)和致心律失常性右心室心肌病(77.8%)。不相符的这几种疾病的原临床和影像学诊断都是原发性扩张型心肌病。致心律失常性右心室心肌病、缺血性心肌病、心肌致密化不全和巨细胞性心肌炎都有特征性的病理形态表现,酒精性心肌病和高血压性心肌病等的病理诊断需要参考临床资料。结论进行心脏移植病例受体心脏的病理学检查有利于提高心脏病的临床和影像诊断的正确率,有扩张表现的心脏病的诊断要排除心脏扩张的继发原因,并要注意与终末期心脏疾病作鉴别。  相似文献   

3.
目的探讨对肥厚型心肌病合并冠心病患者给予心脏CT检查后获得的效果。方法选择2011年04月~2017年01月我院收治的肥厚型心肌病合并冠心病患者60例作为研究对象;临床对所有肥厚型心肌病合并冠心病患者展开心脏CT检查,检查项目主要包括对患者实施冠状动脉CT血管造影检查以及对患者实施延迟强化检查等。之后将所有肥厚型心肌病合并冠心病患者临床实施冠状动脉造影结果以及心脏核磁共振检查结果作为本次诊断依据,最终对临床诊断结果实施回顾性分析。结果在心功能以及左心室室壁厚度方面,CMR方法同CT方法之间表现对比,差异有统计学意义(P0.05)。结论对于肥厚型心肌病患者合并冠心病患者,临床选择心脏CT检查的方法进行疾病诊断,针对患者冠状动脉狭窄程度、患者的心功能以及患者的心室形态等诸多方面可以进行有效了解,针对肥厚性心肌病合并冠心病患者临床疾病的鉴别诊断可以提供准确参考依据。  相似文献   

4.
目的探讨12导联DCG对冠状动脉病变的诊断价值。方法选择98例临床疑诊冠心病行冠状动脉造影检查的患者,同期行12导联DCG检查,将DCG检查结果与冠状动脉造影结果进行对比分析。结果98例患者中经冠脉造影证实有冠脉病变者65例,12导联DCG检测中50例结果阳性,其中有冠脉病变患者40例。12导联DCG诊断冠心病的敏感性72.7%,特异性76.7%,准确性74.5%,随着冠脉病变支数增多,12导联DCG诊断冠心病的阳性率增高。结论12导联DCG对冠状动脉病变具有一定的诊断价值。  相似文献   

5.
心脏移植是终末期心脏衰竭的一种有效治疗手段,然而移植后移植心脏内迅速进行性的血管闭塞,是引起心脏移植后远期死亡或再次移植的重要原因,又被称为心脏同种异体移植物血管病变或移植相关冠状动脉疾病(transplant associated coronary artery disease, TxCAD)。根据国际心肺移植协会登记处统计,TxCAD的发生率为移植后5年32.3%,8年45.7%。形态学上,TxCAD不同于非移植后的“天然”动脉粥样硬化性冠状动脉疾病,其病变更为弥散,病变累及远端的心外膜血管和心肌内的血管;组织学上,则具有同心环状的内膜增生伴少量弹性薄层的特点。由于此种血管病变的弥散性,传统的介入手段仅仅只在病变自然进程的晚期可选择,但效果往往不甚满意;经皮冠状动脉腔内成形术(PTCA)和支架置入术后5年具有68%的高再狭窄率,  相似文献   

6.
18例心肌致密化不全患者的临床特征   总被引:5,自引:0,他引:5  
目的 探讨心肌致密化不全患者的临床特征。方法 回顾分析了18例心肌致密化不全患者的临床表现、心电图、超声心动图等资料,随访(11,4-5)个月。结果 心肌致密化不全占同期确诊的各类原发性心肌病的8,1%。发病年龄1.5~71岁,男性占66.7%,家族性发病2例。14例患者表现为程度不等的心力衰竭,2例患者因心律失常表现为黑喙、晕厥,1例患者发生血栓栓塞,1例患者无症状。心电图均有异常,室性期前收缩7例,心脏传导阻滞和心房颤动各4例。所有患者均通过心脏超声检查确诊,其中17例患者病变局限于左心室,1例局限于右心室,心尖部最常受累(72%),收缩末期非致密化心肌层/致密化心肌层2.3~3.1,15例患者左室射血分数〈50%,室壁弥漫性运动障碍。随诊期间13例患者反复出现心力衰竭,1例患者于确诊后16个月发生心脏性猝死,1例患者因顽固性心力衰竭于确诊后10个月接受心脏移植,1例患者因药物难以控制的心律失常置入埋藏式自动心脏复律除颤器,口服抗凝药的患者未再发血栓栓塞。结论 心肌致密化不全的病情迁延,男性多见,有家族遗传倾向,在原发性心肌病中并不罕见。临床表现主要是心力衰竭、心律失常、血栓栓塞。心脏超声为确诊的首选检查。预后较差,顽固性心力衰竭和致死性心律失常是主要死因,心脏移植、置人埋藏式自动心脏复律除颤器能降低致死(残)率,口服抗凝药能减少血栓栓塞的发生。  相似文献   

7.
目的总结心尖肥厚型心肌病(AHCM)患者的冠状动脉病变特点,并对心尖肥厚型心肌病预后的影响因素进行分析。方法连续人选自2005年1月至2012年8月经超声心动图和(或)心脏磁共振成像确诊为心尖肥厚型心肌病并进行了冠状动脉造影或多排螺旋CT(MDCT)检查的患者共240例,总结其冠状动脉病变特点,并分析冠状动脉病变对AHCM预后的影响。结果74例(30.83%)患者合并冠心病,其中单支病变32例,双支病变25例,三支病变17例;33例经皮行冠状动脉介入治疗(PCI),2例行冠状动脉旁路移植术(CABG),余39例采取单纯药物治疗。23例(9.58%)患者合并冠状动脉肌桥,受累血管均为前降支单支病变;其中1例经皮行冠状动脉介入治疗(PCI),1例行冠状动脉旁路移植术(CABG),余21例采取单纯药物治疗。合并冠心病组(74例)与非冠心病组(143例)比较,前者平均年龄、平均糖化血红蛋白水平及高血压病史的比例均高于后者(P〈0.05);合并冠心病组预后较冠脉正常组差,Cox比例风险回归模型多因素分析发现,左室射血分数(HazardRatio==0.826,95%C10.746—0.915,P=0.001)和左房前后径大小(HazardRatio--1.423,95%CI:1.142-1.773,P=0.002)是影响AHcM合并冠心病预后的独立危险因素。结论应当对高龄、以胸痛主诉、合并高血压病及平均糖化血红蛋白水平均较高的AHCM患者常规进行冠状动脉检查;合并冠心病对AHCM预后有不良影响;LVEF和左房前后径大小是AHCM合并冠心病预后的独立危险因素。  相似文献   

8.
心脏移植的受体选择DaleG.Renlund当考虑心脏移植时,首先要确定患者是否真正必须心脏移植,因为,心脏移植不是根治心衰的方法,只是一种治疗手段,所以,心脏移植前要进行彻底的检查,以确定是否存在可逆性病变,内科治疗或外科手术(不包括心脏移植)有无...  相似文献   

9.
目的:探讨缺血性心肌病患者的临床特征。方法:对92例缺血性心肌病患者的临床和冠状动脉造影资料进行综合分析。结果:合并血脂异常63例(68.5%),高血压59例(64.1%),2型糖尿病27例,有脑梗死病史者22例。所有病例均并发各种心律失常,以传导阻滞和室性心律失常多见。均伴随不同程度的心力衰竭,冠状动脉造影均有冠脉狭窄,73.2%为多支血管病变。结论:缺血性心肌病患者冠状动脉病变广泛,超声心动图和冠状动脉造影对缺血性心肌病诊断具有决定性价值。  相似文献   

10.
<正> 选择合适的供心对确保心脏移植术的成功至关重要。然而,大约有10~20%的受者在等待标准的供心过程中死亡。因此,作者放宽了对供心的选择标准。在1983年7月到1987年2月的三年半中,作者为117例病人进行了121次原位心脏移植。受者在术前诊断为充血性心肌病者74例、终末期冠状动脉病变者36例、已接受瓣膜置换术者5例、心内膜纤维化和右室发育不良各1例。其中4例移植后失败再次移植。在121个供心中,有74个符合经典的供心选择标准,而另外47个供心的入选标准较宽:  相似文献   

11.
BackgroundFew data exist on the characteristics and outcomes of patients with arrhythmogenic right ventricular cardiomyopathy and advanced heart failure who undergo heart transplantation.AimTo explore the pretransplant course and outcomes of patients with arrhythmogenic right ventricular cardiomyopathy after heart transplantation.MethodsThis observational retrospective monocentric study included all consecutive patients with arrhythmogenic right ventricular cardiomyopathy who underwent heart transplantation during a 13-year period (2006–2019) at Pitié-Salpêtrière University Hospital (Paris).ResultsA total of 23 patients with arrhythmogenic right ventricular cardiomyopathy underwent heart transplantation between 2006 and 2019. The median time from diagnosis to heart transplantation was 9 years, and the median age at transplantation was 50 years. At diagnosis, half of the patients had left ventricular dysfunction, 59% had extensive T-wave inversion and 43% had a history of sustained ventricular tachycardia. Only five patients were involved in intensive sport activity. Indications for heart transplantation were end-stage biventricular dysfunction in 13 patients, end-stage right ventricular heart failure in seven and electrical storm in three. Only three patients had pulmonary hypertension, and half of the patients had atrial arrhythmias. The survival rate 1 year after heart transplantation was 74% (95% confidence interval 53–88%). Eight patients experienced primary graft dysfunction needing extracorporeal membrane oxygenation.ConclusionsPatients with arrhythmogenic right ventricular cardiomyopathy who eventually needed heart transplantation mostly exhibited extended disease with biventricular dysfunction at diagnosis. Intensive sport activity did not seem to be a major determinant. Advanced heart failure usually occurred late in the course of the disease. Primary graft dysfunction after heart transplantation was frequent, and should be anticipated. Additional data are needed to identify the optimal timing for heart transplantation and predictors of end-stage heart failure in patients with arrhythmogenic right ventricular cardiomyopathy.  相似文献   

12.
The present report describes a 40-year-old woman with a long history of monomorphic ventricular tachycardia and left bundle branch block. She was treated with various antiarrhythmic agents; ventricular tachycardia ablation was attempted and an automatic implantable cardioverter defibrillator was implanted. Three-dimensional echocardiography clearly demonstrated features of arrhythmogenic right ventricular cardiomyopathy, including marked right ventricular (RV) dilation, decreased RV systolic function and thinning of the RV free wall. Other RV morphological abnormalities included excessive trabeculations and a localized apical aneurysm. Two years later, the patient developed symptoms of congestive heart failure. Despite maximal medical therapy, her clinical condition continued to deteriorate and she was referred for heart transplantation. Results of the pathology of her explanted heart confirmed this rare diagnosis. She presented with an unusual clinical course for arrhythmogenic right ventricular cardiomyopathy, which was complicated by progressive congestive heart failure and ultimately required heart transplantation. Three-dimensional echocardiography identified the structural abnormalities related to this rare disease.  相似文献   

13.
BACKGROUND: The decrease in heart rate variability (HRV) might be related to the hemodynamic status in heart failure. However, HRV in patients with severe isolated right heart failure has not been extensively studied. AIMS: This study compared HRV in patients with congestive heart failure (CHF) and in patients with isolated right heart failure. METHODS: Time and frequency domain analysis of HRV on 24-h ECG recording was assessed in 15 healthy subjects and in two groups of patients with severe heart failure awaiting heart or heart/lung transplantation. These were 15 patients with CHF due to idiopathic dilated cardiomyopathy (IDC) and 10 patients with isolated right heart failure due to primary pulmonary hypertension (PPH). RESULTS: Measurement of HRV were significantly decreased in both groups of patients compared with the control group. Patients with IDC had higher pulmonary capillary wedge pressure than patients with PPH (P=0.04) but lower pulmonary artery pressure and lower pulmonary vascular resistance (PVR) (P<0.0001). However, all the measurements of HRV were significantly lower in patients with IDC than in patients with PPH (range 22-77%, P<0.05 to P<0.01). None of the HRV measurements correlated with filling pressure measurements. CONCLUSIONS: The increase in pulmonary vascular resistance in heart failure is not the main causal factor behind a decrease in HRV.  相似文献   

14.
Cardiac transplantation has become a standard therapeutic option for certain disorders in which poor cardiac output without other surgical options exists in the face of maximized medical therapy. The most common disorder requiring transplantation is dilated cardiomyopathy, although other forms of cardiomyopathy (ie, restrictive cardiomyopathy, arrhythmogenic right ventricular dysplasia/cardiomyopathy, and hypertrophic cardiomyopathy with poor ventricular function) may require transplantation as well. In this review, the current indications and outcomes of heart transplantation in patients with cardiomyopathy are discussed.  相似文献   

15.

BACKGROUND:

Heart transplantation remains the last treatment option for patients with end-stage cardiac disease. Such diseases include ischemic cardiomyopathy, nonischemic cardiomyopathy and other conditions such as arrhythmogenic right ventricular dysplasia, cardiac sarcoidosis and cardiac amyloidosis.

OBJECTIVE:

To review the changes that have occurred over time in the etiology of heart disease in patients requiring heart transplantation, and to compare the clinical and histological diagnoses of explanted hearts from patients with progressive cardiac disease.

METHODS:

The pathological findings of 296 surgically excised hearts over a 20-year period (January 1987 to July 2006) at one institution were examined. Patients were separated into groups based on year of heart transplantation. The tissue was examined to determine the underlying cardiac pathology leading to congestive heart failure. Patient records were reviewed for preoperative clinical diagnoses and other relevant data, including pretransplant endomyocardial biopsy (EMB) results, information regarding left ventricular assist devices and, finally, evidence of disease recurrence in the grafted heart.

RESULTS:

A shift in the underlying etiology was found in patients who underwent heart transplantation from 1992 to 1996, and 1997 to 2001. Between 1987 and 1997, the majority of transplant cases consisted of ischemic cardiomyopathies. From 1997 to 2001, the majority of patients had nonischemic cardiomyopathies, and this trend continued to 2006. A majority of patients with ischemic and hypertrophic cardiomyopathy were diagnosed correctly (96.5% and 82%, respectively) before transplantation. Most patients diagnosed post-transplant with lymphocytic (viral, 15%), hypersensitive/eosinophilic (25%) and giant cell (100%) myocarditis, arrhythmogenic right ventricle dysplasia (100%), cardiac sarcoidosis (83%) and iron overload toxicity-associated cardiomyopathy (100%) had been misdiagnosed in pre-transplantation investigations. Investigations before transplantation did not include an EMB. Of all 296 patients, 51 patients (17%) were misdiagnosed. Excluding the patients with ischemic cardiomyopathy, 46 of 152 patients (30%) were misdiagnosed before transplantation.

CONCLUSIONS:

Although cardiac transplantation is a viable treatment option for patients with a variety of cardiac diseases, accurate diagnosis of patients before transplantation remains a priority. Accurate diagnosis of particular diseases (sarcoidosis, myocarditis, iron toxicity-associated cardiomyopathy and others) allows for proper treatment before transplantation, which may slow down disease progression and improve patient outcomes. Furthermore, it is important to accurately diagnose patients with diseases such as sarcoidosis, amyloidosis and particular types of myocarditis because these can readily recur in the grafted heart. The risk for recurrence must be known to practitioners and, most importantly, to the patient. We strongly recommend the use of EMB if a nonischemic cardiomyopathy is suspected, because the results may alter the diagnosis and modify the treatment strategy.  相似文献   

16.
目的 探讨心壁错构性发育不良的病理形态表现、特点、类型及其与原发性心肌病的关系.方法 以2004年6月至2007年12月底阜外心血管病医院进行心脏移植的92例心脏为观察对象,在心脏离体后立即进行了肉眼观察、测量和摄影记录,并进行了全面的病理组织学观察.结果 心壁的错构性发育见于多种类型的心脏病,且在原发性心肌病中更为多见,差别只是错构的严重程度和分布范围的不同.心壁内存在轻、中度错构不一定就是疾病,但过多的出现能明显影响心脏的结构和功能,只有某种类型的错构性发育较集中或突出时,病理和临床方面才呈现出某一类型的错构性心肌病.错构的范围和程度与原发性心肌病的心脏扩张表现和心力衰竭出现的早晚有一定的正相关趋势.92例中58例为原发性心肌病,34例为非原发性心肌病,原发性心肌病中有25例为错构性心肌病,占原发性心肌病的43.1%.心壁错构性发育异常的病理形态表现主要有:(1)心壁的过度增殖肥厚;(2)纤维/脂肪组织或脂肪/纤维组织替代;(3)心肌细胞的排列紊乱;(4)冠状动脉结构不良等.错构性心肌病一般是一种错构形式为主的多种错构形式组合,相同临床表现的错构性心肌病,其病理基础不完全相同,纤维-脂肪替代为主的多见于扩张型心肌病和致心律失常性右心室心肌病,心肌排列紊乱多伴随于肥厚型心肌病,冠状动脉结构不良常因心肌缺血而扩张.结论 心壁错构性发育不良是心脏结构变异的常见形态表现,只有错构的分布比较密集、弥漫时才表现出某一特定类型的心肌病,错构比较密集、弥漫或伴有严重心肌变性者易表现出心脏扩张和心力衰竭.  相似文献   

17.
We searched for special features in patients with complete and incomplete right bundle branch block diagnosed as having arrhythmogenic right ventricular cardiomyopathy/dysplasia. Whether right bundle branch block is a frequent finding in arrhythmogenic right ventricular cardiomyopathy should be studied. The question is whether special features exist such as T-wave inversions, localized right precordial QRS prolongation and r'/s ratio<1. RESULTS: ARVC could be diagnosed according to ISFC/ESC criteria in 374 patients. CRBBB was found in 22 cases (6%) and iCRBBB was present in 47 cases (12.5%). In CRBBB T wave inversions ≥ V4 was found in 10 cases (n.s.) and r'/s ratio<1 was present in 12 cases (p<0.001). In iCRBBB T wave inversions ≥ V4 was found in 10 cases (n.s.) and ST segment elevation in right precordial leads was present in 19 cases (p<0.005). In all patients with ARVC localized right precordial QRS prolongation was found. Patients with CRBBB have a bad prognosis: 17 of 22 patients developed biventricular heart failure requiring heart transplantation and diuretic therapy. CONCLUSIONS: CRBBB and iCRBBB are infrequent findings in arrhythmogenic right ventricular cardiomyopathy. Complete right bundle branch block is characterized by r'/s ratio<1. There are no significant T wave inversions ≥ V4. Incomplete right bundle branch block is characterized by ST segment elevation in right precordial leads but not by T wave inversions ≥ V4.  相似文献   

18.
磁共振成像在致心律不齐性右室型心肌病的诊断价值   总被引:3,自引:1,他引:2  
目的回顾性分析27例致心律不齐性右室型心肌病(ARVC)的磁共振成像(MRI)表现,探讨MRI在ARVC的诊断与预后判断中的价值。方法按照1994年WHO关于ARVC的诊断标准,2004年10月至2006年6月共27例临床诊断或病理确诊为ARVC(6例行心脏移植术),男21例,女6例,平均年龄37.4(15~67)岁。采用1.5T超导MRI扫描仪对心脏形态(脂肪浸润、房室大小)、功能(室壁局部与整体运动功能)、心肌灌注与心肌存活等方面进行综合评价。结果形态学:88.89%(24/27)的病例MRI提示心肌脂肪浸润,62.96%(17/27)右室壁变薄,62.96%(17/27)右室心尖肌小梁明显粗乱,66.67%(18/27)右室流出道扩张,51.85%(14/27)右室心尖扩张,66.67%(18/27)右室下壁及游离壁扩张,40.74%(11/27)合并右房增大。心脏功能:18.52%(5/27)的病例右室局部运动功能异常,70.37%(19/27)整体运动功能异常,右室平均射血分数(EF)35%。40.74%(11/27)的患者合并左室扩大并室壁收缩运动明显减弱。心肌首过灌注示10.52%(2/19)的患者左室受累,36.84%(7/19)的患者左室和右室壁出现异常强化,提示心肌纤维或胶原变性。右室壁强化区域主要位于右室游离壁和右室流出道肌壁,左室则主要位于左室侧壁,少数合并左室心尖或室间隔,5例左室侧壁异常强化经术后病理证实为纤维组织。仅1例表现为右室流出道增宽,但左室心肌显著变薄,收缩运动明显减弱;有3例右室MRI无阳性表现,其中2例左室侧壁室壁变薄并运动异常,延迟显像为异常强化,另1例表现为类似扩张型心肌病样改变。结论MRI高度的软组织对比与多序列成像可对ARVC进行全面诊断与预后评价,但少数以左室异常表现为主而无明显或仅轻微右室异常的病例,MRI易误诊,其左室侧壁段的纤维化为ARVC相对特征表现。右室整体运动异常、广泛纤维脂肪浸润、合并左室扩张并运动异常为其预后不良的指标。  相似文献   

19.
Endomyocardial biopsy in right ventricular cardiomyopathy   总被引:7,自引:0,他引:7  
Right ventricular cardiomyopathy is characterized by a progressive myocyte loss and fibro-fatty substitution of the right ventricle. The aim of our study was to assess the diagnostic accuracy of right ventricular endomyocardial biopsy. Using an imaging analyser system, histomorphometric parameters of myocytes, interstitium, fibrous tissue and fatty tissue were evaluated on endomyocardial biopsy from 30 patients with arrhythmogenic right ventricular cardiomyopathy, 29 patients with dilated cardiomyopathy and 30 control patients. The percent area of myocytes decreased from 78.10 ± 7.34 in control to 63.39 ± 9.22 in dilated cardiomyopathy (P < 0.05) and to 47.28 ± 15.01 in arrhythmogenic right ventricular cardiomyopathy (P < 0.01). Fibrous tissue increased from 8.10 ± 3.89 in control to 21.80 ± 9.29 in dilated cardiomyopathy (P < 0.05) and to 24.60 ± 11.37 in arrhythmogenic right ventricular cardiomyopathy (P < 0.05). Fatty tissue varied from 0.33 ± 1.44 in control and 0.07 ± 0.31 in dilated cardiomyopathy to 13.30 ± 17.30 in arrhythmogenic right ventricular cardiomyopathy (P < 0.05). Fatty tissue was a feature of arrhythmogenic right ventricular cardiomyopathy (67% of patients vs. 6% of control and dilated cardiomyopathy patients). Diagnostic values typifying arrhythmogenic right ventricular cardiomyopathy, obtained by excluding any overlapping between confidence intervals in the three groups, were: myocytes <44.95%; fibrous tissue >40.38%, and fatty tissue >3.21%, with 67% sensitivity and 91.53% specificity for at least one parameter. In conclusion, a significant difference between arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy and control exists in terms of amount of myocytes, fibrous tissue and fatty tissue. Presence of fatty tissue and fibrous tissue exceeding 3.21% and 40.38%, respectively should be considered highly suspect for arrhythmogenic right ventricular cardiomyopathy in right ventricular endomyocardial biopsy.  相似文献   

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