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1.
目的 评价大动脉调转术(ASO)手术时机对完全性大动脉转位患儿预后的影响.方法 2008年1月-2009年12月共64例完全性大动脉转位(TGA)患儿在本院行一期ASO,其中36例室间隔完整(IVS),28例并室间隔缺损(VSD).各组依手术时年龄分为IVS/early组(≤14 d)和IVS/late组(>14 d),VSD/early组(≤28 d)和VSD/late组(>28 d).回顾性分析各亚组术后围术期并发症及存活情况;随访出院患儿了解其存活情况.结果 各亚组患儿在围术期肺动脉狭窄、低心排出量综合征、吻合口出血、创口愈合不良、膈肌麻痹、呼吸道异常、心律失常及呼吸道感染等并发症发生率比较差异均无统计学意义.3例患儿院内死亡,TGA/IVS组及TGA/IVS组2个亚组院内病死率比较差异无统计学意义(TGA/IVS组:8.3% vs 0,TGA/VSD组:0 vs 7.7%,Pa>0.05).随访13个月,2例患儿院外死亡,TGA/IVS组及TGA/IVS组中2亚组间早期病死率比较差异均无统计学意义(TGA/IVS组:10.5% vs 10.0%,TGA/VSD组:0 vs 22.2%,Pa>0.05).结论 手术年龄不应是影响TGA患儿预后的主要原因,术前机体状态、早产、感染、遵嘱随访是影响TGA患儿预后的重要因素,术前改善机体状态、术后加强随访有助于改善TGA患儿预后,提高其生存率.  相似文献   

2.
目的总结婴儿先天性心脏病(CHD)非择期手术的临床经验,探讨婴儿CHD的手术方法与时机。方法2006年1月至2007年11月共施行婴儿CHD非择期手术81例,平均年龄7.55个月(6d至12个月),平均体重6.89(2.5—10)kg。病种分布:完全性大动脉错位(TGA)7例;完全性肺静脉异位连接(TAPVC)3例;大型室间隔缺损(VSD)合并肺动脉高压(PH)48例;室间隔完整型肺动脉闭锁(PA/IVS)2例;法乐氏四联症(TOF)12例;三房心(CTA)合并PH2例,其中1例同时合并VSD;房间隔缺损(ASD)合并PH6例,其中3例合并VSD,1例合并PDA;ASD合并肺动脉瓣狭窄1例。全部病例均在中低温或深低温低流量或深低温停循环下行心内畸形矫治术。结果79例行心内畸形根治术,2例行姑息手术。死亡5例,死亡率为6.17%。存活76例,术后临床症状均不同程度改善。结论左向右分流合并PH以及某些复杂紫绀型CHD,宜尽早手术治疗。  相似文献   

3.
完全性大动脉转位患儿外科术后早期并发症和近期随访   总被引:1,自引:0,他引:1  
目的 探讨完全性大动脉转位(TGA)患儿术后早期并发症及近期预后情况.方法 对2008年至2009年行手术治疗的71例TGA患儿基本资料进行回顾性分析,并根据室间隔发育情况分为室间隔完整组(TGA/IVS)和室间隔缺损组(TGA/VSD),探讨术后早期并发症及近期预后情况.结果 术后早期出现低心输出量综合征7例(9.9%)、肺动脉狭窄12例(16.9%)、心律失常21例(29.6%)、膈肌麻痹6例(8.5%)、气道异常6例(8.5%)、下呼吸道分泌物培养细菌阳性13例(18.3%);术后院内死亡3例(4.2%).随访68例出院患儿,失访14例(20.6%),余54例(79.4%)随访时间(12.0±7.4)个月,其中4例(7.4%)死亡,3例(5.6%)因肺动脉狭窄需要介入治疗.可随访的57例患儿术后1年存活率87.7%.TGA/IVS组和TGA/VSD组在术后早期并发症和近期存活率方面差异均无统计学意义.结论 TGA患儿术后尽管可能出现一些并发症,但其预后良好,近期存活率较高.  相似文献   

4.
肺动脉闭锁合并室间隔缺损104例诊断分析   总被引:2,自引:0,他引:2  
目的 分析肺动脉闭锁合并室间隔缺损 (PA/VSD)的解剖类型及血流动力学改变 ,探讨其适宜的手术方式。方法 对 1992年 6月至 2 0 0 2年 5月在广东省心血管病研究所儿科住院的PA/VSD患儿共 10 4例 ,采用超声心动图结合心血管造影术 ,确定闭锁的部位、肺动脉的发育情况及血供来源。结果 右室流出道及瓣膜闭锁、有肺动脉总干 31例 ;肺动脉总干闭锁、左右肺动脉有汇合 5 0例 ;左肺动脉闭锁 10例 ,右肺动脉闭锁 8例 ;左右肺动脉均闭锁 5例。肺动脉的血供来源 :大的主肺动脉侧支血管 5 1例 ,动脉导管未闭 2 7例 ,多支小的侧支血管 2 6例。合并畸形有卵圆孔未闭、房间隔缺损、大动脉转位、完全性房室间隔缺损、右室双出口、镜面右位心、右旋心及左旋心。结论 合并室间隔缺损的肺动脉闭锁可发生在不同部位 ,肺动脉的血供来源多样化  相似文献   

5.
目的 总结新生儿重症先天性心脏病的手术治疗以及围手术期处理经验,评价其临床疗效.方法 2008年1月至2010年3月广州市妇女儿童医疗中心手术治疗新生儿先天性心脏病28例,其中2例动脉导管未闭(PDA)患儿行左侧开胸结扎术;1例右室发育不良合并PDA、三尖瓣重度关闭不全患儿行中央分流术;8例室间隔缺损(VSD)患儿行体外循环下VSD修补术;3例主动脉缩窄、1例主动脉弓离断(IAA)患儿行体外循环下主动脉成型术;9例完全性大动脉转位(TGA)患儿中,8例做了Switch手术,其中合并VSD的2例同时做了VSD修补术;1例TGA/IVS PDA行肺动脉banding+中央分流术,7 d后作了SWITCH手术;3例完全性肺静脉异位连接(TAPVC)纠治;重度肺动脉狭窄3例,肺动脉闭锁1例行右室流出道疏通,佰仁思带单瓣牛心包片重建右室流出道;重症法洛四联症、肺动脉发育不良先行中央分流术,3 d后低氧无法缓解,行右室流出道跨肺动脉瓣环补片扩大术.结果 早期(术后30 d内)无死亡病例,晚期死亡2例:1例为心上型TAPVC,术后因膈肌麻痹、乳糜胸、营养不良衰竭死亡,1例为IAA、VSD、PDA合并主动脉瓣下狭窄患儿,术后因持续严重低心排综合征死亡.26例术后痊愈出院.出院时1例VSD患儿术后膈肌折叠有气促,二氧化碳轻度潴留;2例SWITCH患儿术后肺动脉血流速度稍快;1例右室发育不良、PDA、三尖瓣关闭不全中央分流术后三尖瓣关闭不全中-重度;重度肺动脉狭窄、肺动脉闭锁、重度法洛氏四联症右室流出道疏通术后残余肺动脉轻-中度狭窄3例;VSD合并主动脉畸形术后主动脉仍有轻度狭窄.结论 新生儿重症先天性心脏病手术治疗效果满意.适当把握好手术适应症,选择正确的手术方法,精心细致的围手术期处理是手术成功的关键.术后低心排、肺功能障碍、肾功能衰竭的防治是降低手术后病死率的关键.  相似文献   

6.
完全性大动脉转位(Transposition of the Great Arteries,TGA)是小儿常见的紫绀型先天性心脏病之一,按是否合并室间隔缺损,分为室间隔完整型(TGA/IVS)和室间隔缺损型(TGA/VSD)。本院2006年7月至2008年12月采用大动脉调转术(arterial switch operation,ASO)治疗TGA/IVS患儿5例,疗效较好。现报道如下。  相似文献   

7.
目的 总结大动脉调转手术(ASO)围手术期处理方法,提高患儿的手术存活率.方法 2007年1月至2008年3月我科实施ASO术20例.男17例,女3例,年龄14 d~4岁,平均(4.7±3.6)个月.体重3.0~17.0 kg,平均(5.0±3.3)kg.术前诊断包括完全性大动脉转位室间隔完整型(TGA/IVS)14例,完全性大动脉转位合并室间隔缺损(TGA/VSD)5例,右室双出口伴肺动脉瓣下室间隔缺损(Taussig-Bing综合征)1例.结果 住院死亡2例,术后并发症包括低氧血症10例,肺部感染4例,呼吸衰竭二次插管2例,左肺不张1例,房性心律失常1例,室性心律失常1例,伤口愈合不良2例.结论 大动脉调转手术术前应该积极改善患儿缺氧情况,纠正酸中毒,严重缺氧呼吸衰竭者要急诊手术,术后加强左心功能维护,降低肺动脉压力,同时加强呼吸道管理.  相似文献   

8.
完全性大动脉转位矫治手术的围术期处理   总被引:1,自引:0,他引:1  
目的 总结完全性大动脉转位(TGA)矫治手术的围术期处理措施及影响预后的因素.方法 2004年1月-2008年12月行TGA外科矫治术患儿230例(年龄<7岁).根据临床诊断分组:Ⅰ组为室间隔完整的TGA(TGA/IVS,n=73),Ⅱ组为伴室间隔缺损(VSD)的TGA(TGA/VSD,n=118),Ⅲ组为伴左室流出道狭窄的TGA(TGA/LVOTO,n=39).Ⅰ组和Ⅱ组患儿行大动脉调转术(ASO);Ⅲ组患儿行Rastelli手术、改良REV手术和改良Nikaidoh手术.结果 230例患儿中位数年龄为0.3岁(1 d~7岁);中位数体质量为5.0 kg(2.4~23.0 kg).Ⅲ组体外循环和主动脉阻断时间中位数分别为279 min(100~501 min)和191 min(80~281 min).为3组中最长.3组共死亡22例(9.6%).其中Ⅰ组死亡10例(13.7%),2例术前有严重代谢性酸中毒,未行球囊房间隔扩张术,急诊ASO手术后死于低心排出量;3例发生急性左心衰竭死亡;余5例死亡与伴心血管畸形有关.Ⅱ组死亡9例(7.6%),3例突发心律失常死亡;3例二次气管插管后并肺部感染死亡;1例并主动脉弓缩窄,同期手术矫治,手术后发生脑部并发症死亡;2例左心功能训练手术后肺功能较差,急诊行ASO手术后死亡.Ⅲ组死亡3例(7.5%),其年龄均小于本组中位数年龄,死亡原因为手术后长时间低心排出量.16例伴冠状动脉异常,其中8例术后死亡.结论 TGA/IVS患儿手术前球囊房间隔扩张处理并严重酸中毒患儿,明显影响手术结果,体外循环后注意左心功能的维护.TGA/VSD患儿突发心律失常和术后肺内感染是主要死亡原因.TGA/LVOTO患儿手术时间长,低龄患儿手术死亡危险增加.TGA患儿伴冠状动脉异常病死率增加.  相似文献   

9.
目的 探讨肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管的手术方式,评价其临床疗效.方法 2008年7月至2010年8月本院采用单源化手术治疗肺动脉闭锁(PA)合并室间隔缺损(VSD)及重要主肺动脉侧支血管(MAPCAs)患儿8例.男3例,女5例,年龄4~72个月,中位年龄24个月,体重7.5~15.5 kg,平均体重(11.2±2.4)kg.7例采取分期于术治疗,1例采取一期根治手术.结果 全组患儿均存活.随诊6~20个月,1例一期根治手术患儿痊愈;7例分期手术患儿,临床紫绀症状均好转.其中3例肺动脉明显发育,进一步行二期根治手术治愈.结论 单源化手术是治疗肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管的有效方法,分期手术可取得良好的临床疗效.  相似文献   

10.
婴儿及新生儿先心病心内直视手术208例   总被引:1,自引:0,他引:1  
目的 探讨在婴儿、新生儿年龄段先天性心脏病心内直视手术的可行性及疗效评估。方法 对 2 0 8例先心病患儿实施了心内直视手术 ;手术年龄 3d~ 12个月 ,平均 (7.4 1± 3.6 7)个月 ,其中新生儿 17例 ,1~ 3个月 19例 ,3~ 6个月 4 1例 ,6~ 9个月 6 7例 ,9~ 12个月 6 4例。体重 3~ 11kg,平均 (7.4 6± 3.39)kg。男 135例 ,女 73例 ,手术病种 :室间隔缺损合并重度肺动脉高压类 15 5例(74 .5 % ) ,其余为D TGA、PA(IVS)、TAPVC等青紫复杂性先心病。结果 全组手术死亡 12例 ,病死率为 5 .9% ,年龄小、畸形复杂矫治不完全 ,术后低心排 (5例 ) ,呼吸道病变 (4例 )、术后顽固出血 (3例 )是导致死亡的主要因素。结论 手术治疗是拯救婴儿、新生儿高危复杂先心病的积极、有效的手段。随着麻醉、体外循环、监护技术、手术器械材料的进一步改进 ,手术存活率有望进一步提高  相似文献   

11.
目的 回顾总结小于6个月婴幼儿先天性心脏病的外科治疗.方法 2000年1月~2006年12月,手术纠治年龄小于6个月先天性心脏病1831例.包括:室间隔缺损并肺动脉高压453例,完全性大动脉错位214例、法乐四联症119例、完全性肺静脉异位引流106例、右室双出口69例、肺动脉瓣狭窄65例,完全性房室通道55例、动脉导管未闭53例、主动脉缩窄伴室间隔缺损46例、肺动脉闭锁伴室间隔缺损36例、肺动脉闭锁室间隔完整型31例、主动脉弓中断24例、右室双出口伴肺动脉瓣下室间隔缺损22例、其他538例.根据不同病种采取相应的手术方法纠治.结果 手术死亡98例,手术死亡率5.35%.随着手术方法不断改进,手术总死亡率从2000年的8.23%降至2006年的4.91%.随访:完全性大动脉错位术后发生室间隔残余漏1例,肺动脉瓣上狭窄5例,主动脉瓣上狭窄2例,主动脉瓣下狭窄1例,其中再次手术4例,远期死亡1例;完全性肺静脉异位引流心内型4例在术后出现肺静脉回流梗阻,2例死亡,2例再次手术解除梗阻.早期室间隔缺损术后发生残余漏5例,分流量小,不需再次手术;其余病例随访不完整.结论 婴幼儿先天性心脏病的手术处理时间非常重要,危重复杂型先天性心脏病如不早期手术,将失去手术机会,增加术后危险性和死亡率.  相似文献   

12.
We studied the value of routine neonatal electrocardiography (ECG) in the 1st 48 hours of life to diagnose congenital heart disease in 37 neonates with Down syndrome. Twenty-four infants had no clinical evidence of congenital heart disease, had normal ECGs and normal cardiac anatomy on echocardiography. Thirteen children (35.2%) had congenital heart disease. The ECG was normal in seven infants with congenital heart disease: four with atrial septal defect (ASD), two with tetralogy of Fallot (TOF) and one with ventricular septal defect (VSD). A left QRS axis deviation was found in six infants: five with complete atrioventricular septal defect (AVSD) and one with VSD and mitral valve prolapse. However, only the five infants with complete AVSD had a superior QRS axis. Although neonatal ECG detected the presence of complete AVSD in neonates with Down syndrome, it missed an equal number with other heart defects. Echocardiography remains the way reliably to diagnose or exclude congenital heart disease in these infants.  相似文献   

13.
Summary The age distribution of death in all children with congenital heart disease (CHD), who died in a 27-year period in Central Bohemia (population of 1.2 million), and the data on the incidence of CHD in children born in Bohemia (population of 6.3 million) in 1980 were used to calculate the probability of survival of a child born with CHD. Eighty-six percent of these children survived to the first month of life—mostly those with pulmonary stenosis (PS, 99%), aortic stenosis (AS, 95%), ventricular septal defect (VSD, 92%), and atrioventricular septal defect (AVSD, 91%). Seventy-one percent of patients survived the first year of life—mostly those with PS (97%), AS (91%), atrial septal defect (ASD, 89%), VSD (80%), and persistent ductus arteriosus (PDA, 78%). In total, 67% of CHD patients can be expected to survive childhood. The highest survival rates were found in PS (94%), AS and ASD (84%), VSD and PDA (70–80%), and coarctation of the aorta (COA, 68%). The survival rate for the remaining forms of CHD was less than 50%.The highest mortality rate (10% of all children born with CHD) can be expected in the first postnatal week. The lowest survival in the first week was found among those with hypoplastic left heart (HLHS, 39%), double-outlet right ventricle (DORV, 50%), truncus arteriosus (TrA, 57%), pulmonary atresia (PA, 70%), and transposition of the great arteries (TGA, 83%). In addition, total anomalous pulmonary venous connection (TAPVC) and single ventricle had the highest risk of death in the first year of life.  相似文献   

14.
Unilateral agenesis of the pulmonary artery is a rare congenital anomaly that is commonly associated with ventricular septal defect (VSD). Its clinical presentation is diverse, and if left untreated, it can lead to significant damage and even death. Several surgical techniques have been developed for its management, with variable results. We report the case of a 2-month-old infant with agenesis of the left pulmonary artery (PA) and VSD. One-stage primary surgical re-anastomosis was successful in establishing continuity to the main PA and preventing further damage to the heart.  相似文献   

15.
肺动脉闭锁合并室间隔缺损的临床病理分析   总被引:2,自引:0,他引:2  
目的 研究肺动脉闭锁合并室间隔缺损的解剖类型和血流动力学改变,并探讨其适宜的手术方式.方法 回顾性分析233例肺动脉闭锁合并室间隔缺损的造影结果,分析肺血来源、肺动脉发育情况和合并畸形及其与手术预后的影响.结果 233例中中央肺动脉存在,肺血单纯由未闭的动脉导管供应者112例(48.1%),其中1例为双侧动脉导管(0.5%);中央肺动脉和大的主一肺动脉侧支血管(MAPCA)均存在者104例(44.6%);无中央肺动脉,仅有MAPCA供应肺血者17例(7.3%).肺动脉闭锁部位以右室流出道和瓣膜闭锁最多见(48.1%).侧支血管的来源包括直接的主动脉一肺侧支动脉,间接的主动脉一肺侧支动脉和支气管动脉.合并畸形包括心脾综合征、房室连接不一致、心室大动脉连接不一致、多发室间隔缺损、右位主动脉弓、房间隔缺损、左上腔静脉残存、内脏异位症、上下心室、肺静脉异位引流和冠状动脉起源异常等.结论 肺动脉闭锁合并室间隔缺损患者肺血来源多样化,肺动脉发育程度不一,并可合并多种心内、心外畸形,影响手术方法的选掸和手术结果.  相似文献   

16.
Many congenital heart defects with pulmonary valve pathology are repaired or palliated in childhood. It is anticipated that these patients will need subsequent pulmonary valve replacement due to the pulmonary regurgitation or right ventricular dilation/failure that results from their original operation. Interestingly, some patients with prior congenital heart surgery and no congenital pulmonary valve pathology require pulmonary valve replacement in adulthood. The current study analyzed this subset of patients. The pediatric and adult congenital cardiac surgical databases at a large academic center were reviewed from 2001 to 2008 for pulmonary valve replacements. Patients without congenital pulmonary valve disease were identified. Preoperative, operative, and postoperative data were analyzed. Between 2001 and 2008, five patients with congenital heart disease but no pulmonary valve pathology underwent late pulmonary valve replacement. The initial congenital diagnoses were coarctation/ventricular septal defect (n = 3), complete atrioventricular septal defect (n = 1), and anomalous left coronary artery from the pulmonary artery (PA) (n = 1). All five patients had undergone main PA manipulation during their previous operations. Four of these patients had received PA banding and debanding, and one had received a Takeuchi tunnel repair and revision. All the patients underwent pulmonary valve replacement secondary to pulmonary regurgitation and right ventricular dilation. The average time from the last previous operation to the time of pulmonary valve replacement was 20.8 ± 9.1 years. Bioprosthetic valves were used for all the pulmonary valve replacements, and there were no mortalities. Patients who have undergone previous PA manipulation, regardless whether congenital pulmonary pathology is present, may be at risk for pulmonary valve replacement in adulthood. This report describes five cases of patients with a history of congenital heart surgery but no congenital pulmonary valve pathology who required pulmonary valve replacement due to the consequences of prolonged pulmonary regurgitation. Although pulmonary regurgitation may be well tolerated for many years, it is further evidence for the importance of close follow-up assessment and monitoring of young adults with congenital heart disease.  相似文献   

17.
Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely "normal," markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1-1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary.  相似文献   

18.
新生儿小婴儿先天性心脏病围术期肺炎的临床特征   总被引:3,自引:1,他引:2  
目的 总结新生儿小婴儿先天性心脏病嗣术期肺炎临床特征,提高该类型肺炎诊治水平.方法 回顾性分析2006年10月至2009年9月,体重≤5 kg和/或年龄≤3个月病例309例,其中男196例,女113例,包括室间隔缺损206例,房间隔缺损126例,单纯动脉导管未闭18例,中重度肺动脉高压160例,完全性大血管错位37例,肺动脉闭锁11例,完全性肺静脉畸形引流9例,完全型心内膜垫缺损7例,主动脉弓离断5例,右室双出口5例.根据术前、术后呼吸道症状、体温、氧合指数、血白细胞计数、X线胸片等诊断肺炎.结果 全组围术期肺炎患病率58.9%(182/309),总病死率7.1%(13/182),机械通气(47.44±38.0)h,术后ICU(5.2±4.1)d.Logistic回归分析提示早产儿、营养不良、反复感染史、肺高压、复杂先天性心脏病、急诊手术、阻断>60 min、延迟关胸、术后低氧、肺不张、气胸或大量胸腔积液、二次气管插管、插管>4 d等与患病密切相关.结论 新生儿小婴儿围术期肺炎患病率高,临床表现复杂,术前在内科治疗的基础上尽早手术,术后早期诊断,加强综合防治,并发心衰应积极处理.  相似文献   

19.
668例婴幼儿室间隔缺损的手术治疗   总被引:3,自引:0,他引:3  
目的:探讨近5年间外科手术治疗婴幼儿期室间隔缺损(VSD)的结果和经验。方法:对1998--2002年的668例婴幼儿VSD修补术进行总结:年龄1~36个月(平均15-8个月),体重3.0~15.5k(平均8.1kg)。膜周部VSD 562例,干下型VSD 102例.膜周部并肌部VSD4例。术前中度以上肺动脉高压386例(57.8%)。因难治性肺炎或心衰而急诊手术89例(13.3%)。采用中度低温体外循环下手术。结果:全组手术死亡6例,手术病死率为0.9%(6/668):术后主要并发症为肺部感染、心律失常和肺动脉高压症。结论:随着麻醉、体外循环、手术技术和围术期监护处理措施的进步,在婴幼儿期施行室间隔缺损的外科手术已成为安全的治疗方式。  相似文献   

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