时间-空间关联成像联合B-flow在胎儿永存左上腔静脉产前超声诊断中的应用价值

目的探讨时间-空间关联成像(STIC)联合B-flow在胎儿永存左上腔静脉产前超声诊断中的应用价值。方法对产前胎儿常规二维超声心动图可疑或明确心脏畸形的132例胎儿进行B-flow显像及STIC数据采集,观察有无左上腔静脉,并将产前超声诊断结果与产后新生儿超声心动图及引产后胎儿尸体解剖病理检查结果进行对比分析。结果 132例胎儿中120例在B-flow显像模式下STIC数据采集成功,采集成功率为90.9%(120/132);120例STIC数据采集成功的胎儿中,常规二维超声心动图诊断永存左上腔静脉15例(15/20)。B-flow-STIC诊断永存左上腔静脉20例。其中5例伴无名静脉缺如,4例合并心脏严重复杂畸形(2例合并完全性房室间隔缺损,1例合并三尖瓣发育不良,1例合并右心室双出口,1例合并右位主动脉弓,5例合并室间隔缺损),6例为单纯性永存左上腔静脉。B-flow-STIC修正常规二维超声心动图诊断5例;其中2例常规二维超声心动图诊断为房间隔缺损,1例常规二维超声心动图诊断为完全性房室间隔缺损,1例常规二维超声心动图诊断为心内型肺静脉异位引流,均经B-flow-STIC修正诊断为永存左上腔静脉;1例常规二维超声心动图漏诊,B-flow-STIC诊断为永存左上腔静脉引流至左心房。结论 STIC联合B-flow可清晰显示胎儿永存左上腔静脉及其空间位置关系,有利于提高永存左上腔静脉产前超声诊断准确性。     

二维及实时三维超声心动图诊断无顶冠状静脉窦综合征的价值

目的探讨二维及实时三维超声心动图(RT-3DE)诊断无顶冠状静脉窦综合征(UCSS)的临床应用价值。方法回顾性分析8例由手术或心导管造影证实为UCSS患者的超声图像资料,总结UCSS二维及RT-3DE表现。结果 8例UCSS中,Ⅰ型(完全型)3例,Ⅱ型(中间部分型)2例,Ⅲ型(终末部分型)3例。8例UCSS中合并永存左上腔静脉7例,完全型心内膜垫缺损2例,原发孔型房缺1例,继发孔型房缺1例,部分型肺静脉异位引流1例。与手术或心导管造影检查结果对照,二维超声心动图正确诊断UCSS 5例,疑诊2例,误诊为原发孔型房间隔缺损1例;结合RT-3DE后,二维超声检查疑诊病例得以正确诊断,误诊病例经三维成像后诊断为UCSS。UCSS二维超声心动图主要特征:冠状静脉窦(CS)间隔连续性中断,CS扩张,未见正常CS汇入右心房,并常合并其他心内畸形。CDFI血流自左房经CS间隔缺损分流至右房可进一步证实UCSS存在。RT-3DE可多平面、多角度实时观察CS走行及其与左、右心房间的交通,可直观、立体地显示UCSS。结论在二维超声心动图基础上,联合RT-3DE有助于诊断无顶冠状静脉窦综合征。     

超声漏诊误诊无顶冠状静脉窦综合征13例分析

目的分析超声漏诊、误诊无顶冠状静脉窦综合征（UCSS）病例,提高术前诊断准确性。 方法回顾性分析2013年1月至2021年1月华中科技大学同济医学院附属协和医院心外科手术患者中术前超声漏诊和误诊的UCSS病例13例,观察超声图像及图像描述,包括成像质量、重要切面显示情况、图像关键特征等,并与手术结果对照。 结果13例病例中,漏诊4例,包括3例Ⅰa型和1例Ⅱa型。误诊9例,包括将UCSS误诊为原发孔型房间隔缺损（ASD）2例、继发孔型ASD 2例、下腔静脉型ASD 1例,另将1例下腔静脉型ASD、2例右肺静脉异位引流心内型、1例永存左上腔静脉（PLSVC）所致的冠状静脉窦（CS）增宽误诊为UCSS。漏诊病例中2例未扫查CS及PLSVC相关切面,1例留存部分相关切面但不能识别UCSS,1例因声窗差不能显示PLSVC。5例将UCSS误诊为其他疾病者,均未专门扫查CS及PLSVC,仅无意中留存部分切面,且对切面中连续中断、异常血流所处的位置、分流束的走行及形态解读错误。4例将其他疾病误诊为UCSS者,术前均针对CS及PLSVC进行专门扫查,但因CS扩张、移位而忽视其存在,从而误判为CS完全缺失,或因窦壁迂曲、薄弱而回声失落,在附近血流信号干扰下错误解读为窦壁缺损及其分流。 结论UCSS漏诊和误诊与检查者认识不足、扫查手法不熟练、对窦壁与房间隔连续中断及其异常血流解读错误等因素有关。提高UCSS诊断警惕性,加强其与ASD、心内型肺静脉异位引流等的鉴别意识,可提高术前超声诊断准确性。     

超声心动图诊断肺动脉吊索

目的 探讨超声心动图在肺动脉吊索诊疗中的应用价值.方法 分析经CT、心导管造影和(或)手术证实的6例肺动脉吊索患儿的超声心动图表现及诊断要点.结果 6例肺动脉吊索患儿中,超声诊断4例,漏诊1例,误诊1例.6例中3例合并房间隔缺损,1例伴有卵圆孔未闭,2例伴有室间隔缺损,1例伴有动脉导管未闭,1例伴有法洛四联症,1例伴有永存左上腔静脉.结论 超声心动图可探查左、右肺动脉的起始位置及走行,有助于正确诊断肺动脉吊索.     

超声心动图诊断冠状静脉窦区病变

目的探讨超声心动图诊断冠状静脉窦（CS）区病变的价值。方法回顾性分析了13例相关患者的超声心动图诊断,其中9例进行了术后诊断对照。结果11例CS增宽,主要有永存左上腔静脉、完全型肺静脉异位引流、左冠状动脉-冠状静脉瘘等;1例右冠状动脉-右房瘘;1例右冠状动脉假性动脉瘤误诊为冠状静脉窦内实质性团块。结论CS区病变较多,超声心动图显像清晰,诊断信息丰富,应作为首选的检查手段。     

无顶冠状静脉窦综合症的超声心动图诊断

目的 探讨应用超声心动图诊断无顶冠状静脉窦综合症的价值.方法 回顾性分析17例无顶冠状静脉窦病例,将其超声心动图表现与手术结果进行对照分析,总结超声心动图的特征性表现.结果 17例无顶冠状静脉窦病例中,超声心动图正确诊断15例,疑诊1例,漏诊1例,诊断符合率为94%.所有病例中,合并永存左位上腔静脉11例,完全型房室间隔缺损6例,完全性肺静脉异位引流1例,部分型肺静脉异位引流2例,完全性大动脉转位1例,矫正性大动脉转位1例,中央孔型房间隔缺损2例,上腔型房间隔缺损2例.结论 超卢心动图对无顶冠状静脉窦的诊断具有重要价值,可以确诊缺损部位、合并畸形、有无永存左位上腔静脉,可为手术提供重要的信息.     

超声三血管气管切面在胎儿永存左位上腔静脉超声诊断中的应用

目的 探讨超声三血管气管切面（3VT）在胎儿永存左位上腔静脉（PLSVC）超声诊断及病理解剖特征分析中的作用。方法 对8例产前三血管气管切面诊断PLSVC胎儿的声像图及引产后尸检结果进行对照分析。胎儿PLSVC在三血管气管切面超声主要观察：（1）上腔静脉位置。（2）上腔静脉数目。（3）双侧上腔静脉血流方向改变。结果 （1）8例胎儿中7例均可见左位上腔静脉与右侧上腔静脉合并存在为双上腔静脉（DSVC）：其中4例肺动脉和主动脉位置正常,肺动脉左侧及主动脉弓右侧分别可见左、右上腔静脉显示。2例肺动脉和主动脉位置异常,则主动脉弓左侧及肺动脉右侧分别可见左、右上腔静脉显示。此6例胎儿双侧上腔静脉血流方向一致,超声诊断PLSVC,尸检证实为DSVC。1例超声显示为DSVC,但双侧上腔静脉血流方向相反,超声诊断PLSVC,引产后尸检证实为完全性心上型肺静脉异位引流,左侧静脉为垂直静脉。（2）8例中1例上腔静脉位置异常：肺动脉右侧无上腔静脉显示,肺动脉左侧可见上腔静脉,超声诊断PLSVC,尸检证实为右侧上腔静脉缺如、左侧上腔静脉伴心房反位和腹腔脏器反位。（3）8例胎儿均合并各种复杂的心内畸形。其中心脏位置异常4例,右心室及形态学右心室双出口4例,肺动脉狭窄3例,室间隔缺损4例,心内膜垫缺损1例,单心室1例,右心发育不良1例,法洛四联症1例,完全性肺静脉异位引流3例。（4）腹腔脏器反位2例。4例胎儿肺动脉和主动脉位置正常,肺动脉左侧及主动脉弓右侧分别可见左、右上腔静脉显示;2例胎儿肺动脉和主动脉位置异常,在主动脉弓的左侧及肺动脉的右侧分别可见左、右上腔静脉显示, 双侧上腔静脉血流方向一致为DSVC。（5）1例DSVC,但双侧上腔静脉血流方向相反为完全性心上型肺静脉异位引流。结论 3VT可作为胎儿PLSVC诊断切面和方法。在胎儿3VT显示双侧上腔静脉时,双侧上腔静脉血流方向对鉴别永存左位上腔静脉和完全性心上型肺静脉异位引流具有重要意义。肺动脉右侧无上腔静脉显示,肺动脉左侧可见上腔静脉,为右侧上腔静脉缺如型左位上腔静脉。此时要注意观察是否合并心房反位和腹腔脏器反位。     

心脏超声右心声学造影诊断1例上腔静脉发育异常

永存左上腔静脉（PLSVC）合并右上腔（RSVC）缺如为一种非常罕见的疾病，对合并复杂性畸形的患者，术前可通过彩色多普勒心脏超声右心声学造影技术作出诊断，对心导管介入及外科术中处理具有非常重要的意义。     

永存左上腔静脉的声学造影及超声诊断与手术结果对比分析

目的：研究超声心动图及声学造影诊断永存左上腔静脉(PLSVC)的敏感性并探讨提高其检出率的方法。方法：对23例经手术证实的先心病合并PLSVC的患者与经胸、经食道超声心动图(TTE、TEE)及声学造影进行回顾性对比分析。结果：在23例手术证实合并PLSVC的先心病患者中，术前经超声心动图及声学造影检出22例，占96％。结论：多切面的仔细观察及声学造影，多数患者可经超声心动图及声学造影在术前检出，为外科手术提供更多的信息以指导手术。     

单中心儿童永存左上腔静脉合并先天性心脏病的超声心动图分析

目的应用超声心动图分析永存左上腔静脉(PLSVC)患儿合并先天性心脏病种类,提高超声诊断PLSVC及相关心脏畸形的准确率。方法回顾性分析我院2013～2017年行超声心动图检查的212 309例患儿的超声心动图资料及临床资料,其中先天性心脏病患儿28 403例,永存左上腔静脉患儿1676例,分析永存左上腔静脉合并先天性心脏病类型的分布特征及发生率。结果永存左上腔静脉的检出率约0.79%(1676/212 309),其中Ⅰ型永存左上腔静脉最常见约占99.7%(1671/1676)。永存左上腔静脉在先天性心脏病患儿中的检出率约3.0%(866/28 403)。1676例永存左上腔静脉的患儿中,合并先天性心脏心脏病的患儿866例(51.7%),常见的为室间隔缺损202例(23.3%)、房间隔缺损149例(17.2%)、法洛四联症73例(8.4%)、动脉导管未闭58例(6.7%)、主动脉该缩窄及主动脉缩窄复合畸形52例(6.0%)、心内膜垫缺损34例(4.0%)、右室双出口29例(3.3%)。结论永存左上腔静脉常合并先天性心脏大血管畸形;超声心动图检查可准确诊断永存左上腔静脉及其合并畸形,能够为临床制定患儿治疗方案提供可靠依据。     

超声心动图在诊断新生儿期永存左上腔静脉中的价值

目的探讨超声心动图对新生儿期永存左上腔静脉的诊断价值。方法我院产前胎儿超声心动图检查时明确诊断或疑为永存左上腔静脉胎儿共11例,出生后再行超声心动图检查,总结永存左上腔静脉的声像图特征。结果 11例胎儿产后均明确诊断为永存左上腔静脉。超声图像表现为永存左上腔静脉全程可显示,位于主动脉弓左侧,由左颈内静脉与左锁骨下静脉交汇形成,呈"Y"形,近心端与冠状静脉窦相连;永存左上腔静脉血流频谱与右侧上腔静脉相一致。结论超声心动图对新生儿期引流入冠状静脉窦到右心房的永存左上腔静脉有较好的诊断价值。     

胎儿超声心动图在诊断永存左上腔静脉中的应用

目的 总结永存左上腔静脉胎儿超声心动图特征及临床意义.方法 对2008年5月至2011年1月在我院诊断为永存左上腔静脉的33例胎儿超声心动图表现进行总结分析,并与引产后病理或产后超声心动图诊断结果进行对照分析,总结永存左上腔静脉胎儿超声心动图特征及临床价值.结果 33例永存左上腔静脉胎儿中引产后病理或产后超声心动图证实32例,超声表现为:四腔观可见扩张的冠状静脉窦,三血管观在肺动脉左侧可见一圆形血管回声,为永存左上腔静脉,同时可见右上腔静脉变细,追踪走行可见其与冠状静脉窦相连续.其中18例为单发畸形(18/32,56.25%),14例合并其他畸形(7例合并心内畸形,2例合并心外畸形,5例同时合并心内和心外畸形).胎儿超声心动图将内脏反位综合征合并完全型房室间隔缺损误诊为同时合并永存左上腔静脉1例.结论 胎儿超声心动图是诊断永存左上腔静脉的可靠方法.永存左上腔静脉常合并其他畸形,产前超声发现永存左上腔静脉需进一步检查以排除其他合并畸形.     

Transesophageal echocardiography in the diagnosis of the persistent left superior vena cava.

Three patients with suspected persistent left superior vena cava (PLSVC) diagnosed by transthoracic echocardiography underwent single-plane transesophageal echocardiography. In all three patients transverse cuts through right atrium and coronary sinus demonstrated the presence of PLSVC. Agitated contrast injection into the left antecubital vein opacified PLSVC and coronary sinus in all cases. Transesophageal echocardiography is superior to transthoracic echo in the diagnosis of PLSVC and associated cardiac anomalies.     

Partially unroofed coronary sinus and persistent left superior vena cava: intracardiac echocardiographic observation.

Unroofed coronary sinus, a rare congenital anomaly first described by Raghib and colleagues1 in 1965, is a result of an embryologic error involving imperfect or complete failure of development of the left atriovenous fold, which is manifested as a focal (fenestration or partial unroofing of the coronary sinus) or complete absence of the coronary sinus septum. Before the era of echocardiography, precise diagnosis of this anomaly was possible only during surgical procedure or at autopsy. Since the advent of the echocardiography, several studies have reported the usefulness of two-dimensional transthoracic and transesophageal echocardiography in the diagnosis of unroofed coronary sinus. We describe the intracardiac echocardiographic delineation of partially unroofed coronary sinus and persistent left superior vena cava in a patient with atrioventricular nodal reentrant tachycardia. Incidental finding of the dilated coronary sinus during radio frequency ablation of the tachycardia led to the diagnosis of this unusual anomaly.     

无顶冠状静脉窦的超声心动图诊断方法

目的 研究二维超声心动图诊断先天性心脏病一无顶冠状静脉窦(UCS)的思路和方法.方法 回顾分析16例经外科手术证实并成功矫治的UCS病例的超声心动图检查资料,总结超声诊断UCS的思路和方法.结果 16例UCS术前全部由经胸超声确诊,与术中结果对比,超声诊断正确率100%.其中12例为完全型UCS(75%),4例为部分型UCS.12例(75%)合并永存左上腔静脉经UCS入左房.超声诊断要点如下:①在常规扫查冠状窦的切面无法扫查到正常冠状窦;②房水平分流,CS造成左、右心房之问的分流通道,必有房水平分流,且右房侧分流口位于冠状静脉窦口处,至于分流方向取决于合并的其他畸形;③常合并左上腔静脉经UCS入左房;④由房水平分流引起的心腔结构及血流动力学改变.结论 通过对UCS的结构及血流动力学特点的分析,可以建立有效准确的超声心动图诊断方法.

Abstract：

Objective To conclude the diagnostic methodology of unroofed coronary sinus (UCS) by two-dimensional echocardiography(2DE). Methods By analyzing the echocardiographic results of 16 UCS patients who were diagnosed by 2DE and confirmed by operation, the diagnostic methodology of UCS by 2DE was summarized. Results Sixteen patients with UCS were involved in this study. Among them, 12 cases were diagnosed as complete UCS,others were partial UCS(PUCS). Twelve patients were complicated with persistence of left superior vena cava (LSVC) that was connected to left atrium (LA) through UCS. All of preoperative diagnosis conducted by 2DE were finally confirmed to be consistent with the results of operation. With the analysis of acquired echo images,key points of diagnosis were concluded as follow: 1) normal coronary sinus (CS) could not be detected in the routine 2DE views referring CS. PUCS showed partial absence of CS roof,while complete UCS displayed as total absence of CS. 2)Inter-atrial shunt would definitely be found in UCS and the opening to right atrium must be coronary sinus orifice. The shunt direction was depended on the combined cardiac malformations. 3) With the occurrence of UCS,LSVC would be in junction with LA through UCS. 4) The inter-atrial shunt resulted in cardiac morphologic and hemodynamic changes. Conclusions Better understanding of the anatomic, morphological and hemodynamic characteristics of UCS would greatly contribute to accurate diagnosis on UCS.     

Clinical significance of persistent left superior vena cava diagnosed in fetal life.

OBJECTIVES: To determine the prevalence and clinical significance of persistent left superior vena cava (PLSVC) in fetuses with and without cardiac and extracardiac anomalies. METHODS: Charts and recorded images were reviewed from high-risk patients who underwent fetal echocardiography between January 2000 and December 2005. This retrospective study included 54 fetuses with confirmed PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Associated congenital heart defects (CHDs), extracardiac abnormalities, including first trimester nuchal translucency (NT) thickness, and fetal/postnatal outcome were analyzed. RESULTS: Of 5,737 referrals, 5,233 had a normal heart, and PLSVC was observed in 10 of these fetuses (0.2%; Group 1). CHDs were present in 504 and PLSVC was observed in 44 of these cases (9%). In the latter group, 18/44 (41%) fetuses had heterotaxy syndrome (Group 2) where the most common structural heart defects were atrioventricular septal defect and double-outlet right ventricle. Fetuses without heterotaxy syndrome (Group 3) accounted for 26/44 (59%) cases of CHDs associated with PLSVC. In this group of fetuses the most common CHDs were left outflow tract obstructive defects and conotruncal anomalies. Increased NT was observed in 29%, without differences among the three groups. The survival rates among fetuses in Groups 1, 2 and 3 were 100%, 44% and 50%, respectively. After excluding patients who underwent pregnancy termination, there were no significant differences in the survival rates among the groups, probably due to the small size of the samples. CONCLUSIONS PLSVC is associated with CHDs. The identification of PLSVC should prompt a thorough examination of the fetus to identify additional cardiac and extracardiac anomalies. The prognosis of affected fetuses largely depends on whether or not the PLSVC is associated with a CHD.     

Persistent Left Superior Vena Cava Diagnosed by Bedside Echocardiography

Background: Persistent left superior vena cava (PLSVC) is a congenital anomaly with an estimated incidence of 0.3–0.5% in the normal population. Its usual discovery is often made by an abnormally positioned catheter inserted in the left subclavian or left jugular vein. In this situation, an easy bedside approach to confirm an anatomic variation in the central venous system is helpful. In the majority of cases, the PLSVC drains to the coronary sinus. Objective: To describe the contribution of bedside echocardiography in diagnosing the unstable patient in whom there is suspicion of a PLSVC. Case Report: A 29-year-old man underwent an emergent laparotomy for multiple intra-abdominal abscesses. Postoperatively, after insertion of a central line catheter through the left subclavian vein, a chest X-ray study showed the tip of the catheter in a left paramediastinal position instead of crossing the midline to the superior vena cava. A PLSVC was suspected. The patient was hemodynamically unstable; therefore, a bedside non-invasive confirmation of the diagnosis of PLSVC was preferred. A transthoracic echocardiography study was performed after injection of agitated saline (creating air-filled microbubbles by shaking saline solution in a syringe), which showed that the coronary sinus was opacified, confirming the diagnosis of a PLSVC. Conclusion: In this brief report, we describe the contribution of echocardiography to the diagnosis of a PLSVC. Echocardiography is a reliable and easy diagnostic tool that allows a bedside approach in a patient in whom there is suspicion of a PLSVC, without administration of radiographic contrast.     

产前超声诊断胎儿永存左上腔静脉

目的 探讨产前超声诊断胎儿永存左上腔静脉的临床价值.方法 回顾性分析21例经胎儿超声检查诊断为永存左上腔静脉的超声资料与随访结果,分析总结胎儿永存左上腔静脉各切面异常声像图特点.结果 12例胎儿经尸体解剖证实为永存左上腔静脉,胎儿永存左上腔静脉的主要声像图特征是胎儿心脏三血管切面观,肺动脉左侧多一条血管.冠状静脉窦扩张有助于诊断.结论 产前超声检查是诊断永存左上腔静脉的首选方法,有重要临床意义.     

永存左上腔静脉的平片特征探讨（附34例分析）

目的：评价永存左上腔静脉的影像学诊断价值。方法：经心导管及造影证实的先天性心脏病合并左上腔静脉34例，男16例，女9例，年龄1－27岁。其中32例有手术证实（病变组）。分析其X光平片上纵隔的密度、外型。随机抽取25例先天性心脏病未合并左上腔静脉者作对照分析（对照组）。结果：病变组X光平片示于主动脉弓之上左缘有带状或新月形血管阴影及形成上纵隔呈“V”形增宽阴影18例（52．9％），对照组4例假阳性（16％）。结论：X光平片对左上腔静脉有重要的诊断价值，结合导管和造影可明确诊断。