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大血管错位的相关文献在1990年到2021年内共计83篇,主要集中在内科学、外科学、儿科学 等领域,其中期刊论文82篇、会议论文1篇、专利文献41226篇;相关期刊36种,包括中国医疗设备、中国医学影像技术、中国医学影像学杂志等; 相关会议1种,包括2003年全国中青年心外科医师先心病外科学术会议等;大血管错位的相关文献由243位作者贡献,包括徐志伟、苏肇伉、丁文祥等。

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大血管错位

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  • 徐志伟
  • 苏肇伉
  • 丁文祥
  • 郑景浩
  • 刘锦纷
  • 胡盛寿
  • 张海波
  • 李守军
  • 刘迎龙
  • 沈向东
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    • 张秉权; 刘永熙; 邓宇; 曾庆思
    • 摘要: 目的:研究婴幼儿圆锥动脉干畸形合并冠状动脉异位起源各类型发生率、构成比,通过回归分析探讨圆锥动脉干畸形合并冠状动脉脉异位起源的MSCT分类对手术复杂程度的预判能力.方法:回顾性分析520例经手术证实的婴幼儿圆锥动脉干畸形,所有患儿均在广东省妇幼保健院行MSCT心脏成像并记录圆锥动脉干畸形、冠状动脉异位起源类型及数量.对不同类型圆锥动脉干畸形合并冠状动脉异位起源的发生率及构成比分别采用行×列Pearson卡方检验进行比较,使用Logistic回归对手术复杂程度相关指标的预测能力进行分析,与手术结果对照探讨术前MSCT心脏成像诊断对手术复查程度的预测能力.结果:婴幼儿圆锥动脉干畸形共520例,合并冠状动脉异位起源者91例,平均发病率为17.5%,其中肺动脉闭锁(PA)23例,大动脉转位(TGA)27例,法洛氏四联症(TOF)11例,永存动脉干(PTA)9例,右室双出口(DORV)21例.在本组圆锥动脉干畸形合并不同类型冠状动脉异位起源中单冠状动脉(SCA)最多,占61.53%,其次为对侧冠状动脉或无冠窦起源(OO)占23.07%,冠状动脉多发起源(MO)9.89%,冠状动脉高位起源(HO)4.39%,冠状动脉肺动脉起源(PO)最少,占1.09%.采用行×列Pearson卡方检验,卡方值为27.859,P=0.033,说明不同类型圆锥动脉干畸形的冠脉异位起源构成比不全相同即不同类型圆锥动脉干畸形与冠脉异位起源有关联性.本研究冠状动脉异位起源发生率由高到低依次为PTA(39.13%)>TGA(28.12%)>DORV(22.10%)>PA(17.16%)>TOF(6.35%).Logistic回归显示右心室双出口、大动脉转位、对侧冠状动脉或无冠窦起源联合对手术复杂程度有最好的预测效能.结论:圆锥动脉干畸形合并冠状动脉异位起源的MSCT诊断可有效预测手术复杂程度;当心脏MSCT诊断大动脉转位或者右心室双出口时,因涉及动脉调转术而致手术复杂程度增加,如果合并单冠状动脉或对侧冠状动脉异位起源,近端冠状动脉的游离、松解难度增大致动脉调转术中冠状动脉移植失败的风险增加,影响预后;术前必须明确圆锥动脉干畸形类型及伴发冠状动脉异位起源的类型,详细了解冠状动脉起源及其近段走行状态,降低冠状动脉移植风险及改善预后.
    • 解珺淑; 张晓红; 尹秀菊; 闫亚妮; 裴秋艳; 严荔煌; 张璘; 刘捷; 刘刚; 张慧丽
    • 摘要: 目的 探讨孕期超声检查发现的完全型大动脉转位(D-TGA)胎儿的产前诊断、孕期及围产期一体化管理方法及其预后.方法 回顾性分析2014年1月至2019年6月在北京大学人民医院孕期超声检查发现的19例D-TGA胎儿的产前诊断情况、孕期及围产期一体化管理情况及预后.结果 5年余,孕期超声检查诊断的胎儿D-TGA的发生率为0.12%(19/16028).19例D-TGA胎儿中,单纯D-TGA7例(7/19),D-TGA合并室间隔缺损7例(7/19),D-TGA合并其他心内畸形5例(5/19).19例D-TGA胎儿中合并心外畸形2例(2/19),合并胎儿生长受限1例(1/19),胎儿颈部透明层增厚3例(3/19).19例超声检查发现的D-TGA胎儿中,18例(18/19)进行了胎儿或新生儿的染色体核型分析,发现染色体异常2例,均于孕中期终止妊娠.对D-TGA胎儿进行孕期及围产期一体化管理和多学科联合诊治,9例(9/19)孕中期引产,10例(10/19)足月分娩,分娩孕周为(38.3±0.7)周,其中产科因素剖宫产术分娩6例(6/10),阴道分娩4例(4/10).10例新生儿出生时血氧饱和度为(69.2±11.3)%,根据出生时情况予以常压吸氧或前列地尔6.00~13.00ng·min-1·kg-1泵入,新生儿转出时血氧饱和度为(77.8±6.7)%.10例患儿除1例失访外,其余9例均进行手术治疗,手术时间为出生后(21.8±22.1)d,8例一次手术完成,1例行二次手术.9例手术治疗的患儿随访至今均预后良好.结论 孕期超声检查发现的D-TGA胎儿应行产前诊断,并进行个体化评估和孕期及围产期一体化管理.胎儿D-TGA并非剖宫产术指征,患儿出生后必要时药物维持动脉导管的开放,通过手术可获得良好预后.
    • 叶莉芬; 范勇; 张泽伟; 吴秀静; 舒强; 林茹
    • 摘要: Objective To summary the clinical experience of ECMO for failure to separate from bypass after arterial switch operation of TGA.Methods 8 TGA patients (6 boys and 2 girls,aged 1 day to 3.5 years and weighing 2.7-11.0 kg,3 VSD intact and 5 with VSD,others complicated malformation including COA,left ventricular outtract stenosis) were treated with VA ECMO owing to failure to separate from bypass caused by low output syndrome after ASO between July 2007 and June 2016.We collected the medical records and analyzed the indication,management and complication of ECMO for this patient population.There were two stages of ECMO supporting for low output after ASO,The first stage was to improve tissue perfusion and correct inner environment by high flow supporting,and the second stage was ventricular function training.ECMO was weaned when the blood pressure was more than 60 mmHg and the difference value of systolic pressure and diastolic pressure was 15-20 mmHg under medium dose inotropics supporting.Volume infusion was limited strictly during ECMO.Results The running time were 22-300 h.5 patients were weaned from ECMO successfully and 4 patients discharged to home.The long-term follow-up of echocardiography indicated normal cardiac function in 3 patients.1 older child had left cardiac failure again after weaning from ECMO 12 days later;he was supported by LVAD subsequently.LVAD was weaned after 236h supporting,unfortunately,He died from cardiac failure 50 days after LVAD weaning.3 patients could not wean from ECMO and died.The main complications were bleeding and pericardial tamponade.Conclusion VA ECMO was effective treatment for failure to separate from bypass after switch operation of TGA.The high mortality was seen in patients with intramural coronary arterial.The myocardial structure perhaps changed secondary in older TGA children,ECMO and LVAD can be used as short-term circulatory transition to artificial heart or transplant.Bleeding was the main complication of this population;surgical hemostasis and accurate coagulation management were the guarantee for successful ECMO running.%目的 总结体外膜肺氧合(ECMO)救治大动脉转位调转术后脱机困难的临床经验.方法 回顾2007年7月至2016年6月8例调转术后脱机困难实施ECMO的大动脉转位患儿的临床资料,分析该类患儿的ECMO适应证、运行管理、主要并发症及相应的处理.全组男6例,女2例,年龄1天~3.5岁,体质量2.7~11.0 kg.室间隔完整型大动脉转位3例,室间隔缺损型大动脉转位5例,主动脉弓缩窄、左心室流出道狭窄、肌部室间隔缺损各1例.因术后难以脱离体外循环(低心排血量综合征6例、低心排血量综合征合并肺动脉高压2例),从传统体外循环转为静脉-动脉模式ECMO.结果 ECMO运行22~ 300 h.5例成功撤离,其中4例出院,1例撤离后12天再次左心衰,左心室辅助装置辅助236 h后撤离,50天后并发大量胸腔积液及乳糜胸死亡.3例心功能不能恢复无法撤离ECMO死亡.出血、心包压塞是主要并发症.结论 ECMO是大动脉转位患儿调转术后低心排血量综合征脱机困难的有效辅助治疗手段.解剖畸形纠正满意,冠状动脉走行正常者脱机成功率高且预后良好,壁内型冠状动脉者疗效差.出血是该群体ECMO期间的最常见并发症,严密外科止血和精准的出、凝血管理是保证辅助循环顺利运行的关键.
    • 周浩; 任彦; 张惠锋; 叶明; 陈伟呈; 陶麒麟; 贾兵
    • 摘要: 目的 总结大动脉调转术治疗Taussig-Bing畸形的经验及远期结果.方法 2001年1月至2015年12月,复旦大学附属儿科医院心血管中心行大动脉调转术治疗57例Taussig-Bing畸形患儿,中位年龄63(37.5~88.5)天,中位体质量4.1(3.4~5.0)kg.合并主动脉弓病变29例(50.9%),冠状动脉异常走行23例(40.3%).根据是否合并主动脉弓病变分为A组29例,合并主动脉弓病变;B组28例,单纯Taussig-Bing畸形.根据手术时间段,2001-2008年27例(组1),2009-2015年30例(组2).结果 住院死亡7例,占12.3%(7/57),A组4例(13.8%,4/29),B组3例(10.7,4/28),P>0.05;组 1死亡6例(22.2%,6/27),组2死亡1例(3.3%,1/30),P0.05),group 1 and group 2 was 22.2%, 3.3%(P<0.05) respectively, follow up was complete in 47 patients with a mean follow-up of(6.2±3.5) years , three patients lost, there was no late mortality, the actual survival at 1, 5year was 87%, 87%, respectively.Reintervention was required in 10 patients(21.3%), the aorta-PA valve diameter ratio was a risk factor for reintervention(group A P=0.02, group B P=0.04) ,and 1,2,5year free of reintervention was 95.6%, 86.6%, 77.2%, respectively.Conclusion The ASO approach can be applied to Taussig-Bing anomaly with acceptable mortality , and it is the procedure of choice at our institution.One stage to repair TBA with aortic arch abnormalities did not influence outcomes.The aorta-PA valve diameter ratio<0.5 was a risk factor for reintervention.
    • 龙湘党; 王莉萍; 赵康; 申建华; 敖琨; 姚穗
    • 摘要: 目的 探讨超声心动图在胎儿圆锥动脉干畸形诊断中的价值.资料与方法 回顾性分析45例经超声心动图诊断为胎儿圆锥动脉干畸形的声像图表现及随访资料.结果 超声诊断胎儿法洛四联症13例,右心室双出口10例,大动脉转位14例,永存动脉干8例.40例引产并经病理解剖证实,5例经产后超声心动图和手术证实.1例法洛四联症并肺动脉闭锁误诊永存动脉干,1例右心室双出口误诊为大动脉转位.结论 超声心动图在胎儿圆锥动脉干畸形的诊断和鉴别诊断中具有较大的临床价值.
    • 高仕君; 聂宇; 廉虹; 胡盛寿
    • 摘要: Objective: To establish a mice model of congenital heart disease transposition of great arteries in order to provide a research reference for the occurrence and development of transposition of great arteries. Methods: A total of 20 pregnant ICR mice at 8-10 weeks of age were divided into 2 groups: Control group, the mice received a single dose of DMSO 70 mg/kg at 8.5 days of gestation,n=5 and Experiment group, the mice received a single dose of all-trans retinoic acid 70 mg/kg at 8.5 days of gestation,n=15. All animals were treated for 18 days and then the embryos were taken to observe cardiac morphology under stereomicroscope. Results: Compared with Control group, Experiment group had obviously increased occurrence rates of premature delivery, abortion and embryo absorption, and 61.2% phenotype for transposition of great arteries; meanwhile, combining with non-heart defect phenotypeas exophthalmos and spinal malformation. Conclusion: All-trans retinoic acid may induce transposition of great arteries in mice embryos, which is a feasible animal model in experimental research.%目的:尝试建立一种先天性心脏病大动脉转位小鼠模型,为探讨该类疾病发生、发展的相关机制提供条件。方法:20只8~10周大的SPF级ICR孕鼠随机分为对照组(n=5)和实验组(n=15)。实验组孕鼠在E8.5天给予单次剂量全反式视黄酸(70 mg/kg)腹腔注射,对照组孕鼠在E8.5天给予单次剂量二甲基亚砜(70 mg/kg)腹腔注射。注射完毕后继续饲养至E18天后取出胚胎,在体式显微镜下观察心脏表型。结果:与对照组相比,实验组胚胎的早产、流产、胚胎吸收的发生率显著增加;大动脉转位表型明显,发生率为61.2%,同时合并突眼、神经管畸形等多种心外表型发生。结论:全反式视黄酸可以诱导小鼠胚胎发生大动脉转位,是一种可行的疾病动物模型。
    • 刘承虎; 苏俊武; 李志强; 范祥明; 陈焱; 贺彦; 刘迎龙
    • 摘要: 目的 总结年龄大于6个月的伴室间隔缺损合并重度肺动脉高压完全性大动脉转位患儿诊断性治疗-根治性手术策略的应用经验及术后效果,探讨手术指征.方法 2010年1月至2011年10月手术治疗17例伴室间隔缺损合并重度肺动脉高压完全性大动脉转位患儿,男13例,女4例.中位年龄1.2岁,其中0.5岁~<1.0岁6例,≥1.0岁~<3.0岁3例,≥3.0岁8例.合并动脉导管未闭6例,房间隔缺损5例,二尖瓣关闭不全2例,肺动脉瓣轻度狭窄2例.术前均行超声心动图检查,冠状动脉CT检查11例,右心导管检查3例.全组均行诊断性治疗2~4周,静吸复合麻醉低温体外循环下行大动脉调转术(ASO),术后残留肺动脉高压者继续予肺动脉高压靶向药物治疗.结果 全组无手术死亡.术前经诊断性治疗动脉氧饱和度提高10% ~21%,肺动脉平均压下降10 ~20mmHg(1.33 ~2.67kPa).随访6~32个月,平均11.2个月.随访期间1例死于食物中毒致急性腹泻、电解质紊乱和心律失常,余患儿至最终随访日均生存.术后6例(35.29%)残余肺动脉高压,年龄均≥3岁,肺动脉高压靶向药物治疗6 ~20个月后,肺动脉压力明显下降.结论 大于6个月的伴室间隔缺损肺动脉高压完全性大动脉转位患儿经诊断性治疗后可以选择性实施根治性手术(ASO),效果良好.%Objective To analyze and summarize the applicative experience and operative effective of the diaguostictreatment-repair strategy in the transpossion of great arteries(TGA) infants with ventricular septal defect and severe pulmonary hypertension more than 6 months.Methods From January 2010 to October 2011,17 TGA cases with ventricular septal defect and severe pulmonary hypertension.There were 13 male and 4 female.≥0.5-< 1.0 years old 6 cases,≥ 1.0-< 3.0 years old 3,≥3.0 years old 8 cases.Combine anomalies: patent ductus arteriosus in 6 cases,atrial septal defect in 5 cases,valve insufficency in 2 cases.All preoperative cases were performed echocardiography,right-sided heart catheterization 3 cases,coronary CT examinationll cases.After diagnostic-treatment 2-4 weeks,all cases performed arterial switch operation under compound intravenous and inhaled anesthesia.Results No operative death.After diagnotic-treatment,SPO2 improved 10%-21%,and mPAP decreased 10-20 mm Hg.Follow-up 11.2 (6,20) months,one dead.Postoperative residual pulmonary arterial hypertension in 35.29%,6/17cases,all of them were ≥3 years old.Continue to pulmonary arterial hypertension targeted drugs treatment for 6-20 months later,pulmonary artery pressure decreased obviously.Conclusion The TGA infants with ventricular septal defect pulmonary arterial hypertension more than 6 months,can be selectively performed arterial switch operation under went diagnostic-treatment-repair strategy,continue to pulmonary arterial hypertension targeted drug therapy postoperation,the effect is good.
    • 李世国; 戴汝平; 蒋世良; 吕滨; 韩磊; 宋会军; 张戈军; 金敬琳; 凌坚; 郑宏
    • 摘要: 目的 探讨MSCT在上腔静脉窦型房间隔缺损中的诊断价值.方法 回顾性分析经MSCT诊断的20例上腔静脉窦型房间隔缺损患者的临床、X线胸片、经胸超声心动图等资料,及该病伴发畸形的MSCT解剖征象.结果 患者的上腔静脉窦型房间隔缺损均位于房间隔后上方,直径9.1~34.1mm,平均(17.1±5.8)mm,均伴有右上叶肺静脉异常连接于上腔静脉.3例伴永存左上腔静脉,1例老年患者同时合并冠状动脉左前降支狭窄,另1例老年患者行右心导管检查提示重度肺动脉高压.6例行外科手术治疗,术中所见解剖异常与CT一致.结论 对比剂增强MSCT横轴面图像结合VR三维重组可以明确显示上腔静脉窦型房间隔缺损及伴发畸形的解剖特征,对于该病的确诊及外科手术方案的制定具有较高的临床应用价值.%Objective To evaluate the effectiveness of MSCT in the diagnosis of superior sinus venosus atrial septal defect.Methods The MSCT features of superior sinus venosus atrial septal defect in twenty cases were evaluated retrospectively.The following data were recorded:the size and location of sinus venosus atrial septal defect,the anatomy of pulmonary vein,including number of anomalously draining pulmonary veins and their site of drainage,and associated anomalies.Results In all patients,the superior sinus venosus atrial septal defect locates in the extraseptal wall,which normally separates the right upper pulmonary vein from superior vena cava(SVC).And anomalous connection of right upper pulmonary venous and SVC was identified in all the patients.The mean value of the defect diameter was ( 17.1±5.8) mm.Left superior vena cava was identified in 3 patients.In an elderly patient,left anterior descending branch of coronary artery presented significant stenosis.And in another elderly patient with large atrial septal defect,severe pulmonary hypertension was identified by cardiac catheterization.MSCT findings of superior sinus venosus atrial septal defect in 6 cases were finally confirmed by surgical operation.Conclusions Contrastenhanced MSCT was a useful technique for the diagnosis of superior sinus venosus atrial septal defect,which accurately displayed the anatomical characteristics of the associated malformations for preoperative evaluation.
    • 熊德生
    • 摘要: 观察康斯特保护液(HTK液)对心脏手术中长时间缺血心肌的术后影响.方法:28例完全性大动脉转位手术患者随机分为两组.HTK液组灌注HTK液,St.Thomas液组灌注冷血停搏液.两组分别于送返ICU后1h取静脉血3 mL,行心肌肌钙蛋白、肌酸激酶同工酶、血肌红蛋白检测.并记录临床效果观察指标:体外循环停止时正性肌力药评分、术后留置气管导管时间、ICU治疗时间、死亡率.结果:两组病例术后各项生化检测指标差异均无显著性(P> 0.05);且体外循环停止时正性肌力药评分、术后留置气管导管时间、ICU 治疗时间、死亡率差异亦无显著性(P>0.05).结论:在需长时间阻断的新生儿和婴幼儿心脏手术中,HTK液和冷血停搏液对术后的影响无明显差异.
    • 韦华; 邓劲松; 张日英
    • 摘要: 目的探讨大动脉调转术的麻醉管理方法。方法 20例复杂先心病婴儿,在气管插管全麻、体外循环下行大动脉调转术,麻醉采用间断予芬太尼、咪达唑仑和维库溴铵维持,主动脉开放后给泵入多巴胺、米力农等血管活性药物,维持血流动力学稳定。结果全组麻醉平稳,开放主动脉后心脏均自动复跳,体外循环转流时间126~392(247.1±46.3)min,主动脉阻断时间76~201(129.5±34.2)min,术后呼吸机支持时间23~342 h。术后并发低心排综合症5例,延期关胸5例,低氧血症8例,肺部感染、胸腔积液各2例,急性肾功能衰竭3例。除死亡3例(15%)外余均痊愈出院。结论大动脉调转手术麻醉危险性高,术中缩短动静脉穿刺时间、维持血流动力学平稳,重视低氧血症处理是麻醉管理重点。
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