三血管气管切面联合弓降部冠状切面对主动脉弓及分支畸形的产前诊断应用价值

目的探讨三血管气管切面联合弓降部冠状切面对诊断主动脉弓及分支畸形的产前超声价值。方法回顾性分析50例主动脉弓异常胎儿的超声心动图特征,并与产后超声及病理结果进行对照,分析不同类型主动脉弓异常在三血管气管切面及弓降部冠状切面的超声表现。结果所有病例在三血管气管切面及弓降部冠状切面上均有特异性超声表现。其中主动脉弓缩窄8例,有6例表现为不对称的"V"形及"Y"形,2例表现为"V"形及"Y"形结构消失;主动脉弓离断11例,表现为"V"形及"Y"形结构消失;右位主动脉弓伴左锁骨下动脉迷走和左位动脉导管8例,表现为主动脉弓位于气管右侧,包绕气管的"U"形血管环,以及发自降主动脉的迷走左锁骨下动脉;左位主动脉弓伴右锁骨下动脉迷走和左位动脉导管6例,表现为主动脉弓位于气管左侧,包绕气管的"C"形血管环,以及发自降主动脉的迷走右锁骨下动脉;镜面右位主动脉弓12例;表现为主动脉弓位于气管右侧,主动脉弓发出的第一分支均为左无名动脉;双主动脉弓5例,表现为包绕气管的"O"形血管环以及位于降主动脉两侧的左右双弓。结论三血管气管切面是诊断胎儿主动脉弓及分支畸形的有效切面;弓降部冠状切面是鉴别诊断主动脉弓异常类型的重要辅助切面。     

二维超声心动图联合时间-空间复合成像对胎儿Berry综合征的诊断价值

摘 要 目的 通过总结二维超声联合时间-空间复合成像（STIC）技术在胎儿Berry综合征诊断中的超声心动图图像特征，探讨STIC技术在诊断过程中的临床应用价值。方法 回顾性分析8例产前超声诊断为Berry综合征胎儿的二维超声、彩色多普勒超声及STIC图像，总结胎儿Berry综合征超声心动图表现。结果 8例Berry综合征胎儿均表现为主-肺动脉间隔缺损（Ⅰ型3例、Ⅱ型5例）、主动脉弓离断（A型6例、B型2例）及右肺动脉起源于升主动脉；3例为孤立性Berry综合征，5例合并其他心内结构异常（2例合并永存左上腔静脉，2例合并右位主动脉弓并迷走左锁骨下动脉，1例合并右室双出口）。8例患儿STIC图像清晰显示了主动脉弓离断位置及左、右肺动脉起源。结论 二维超声联合STIC可准确诊断胎儿Berry综合征，具有重要的临床应用价值。     

先天性血管环的超声心动图诊断价值及漏诊分析

目的评价彩色多普勒超声心动图对先天性血管环(CVR)的诊断价值,分析超声心动图漏诊的原因,旨在提高其对CVR诊断的准确率。方法回顾性分析103例经外科手术证实为CVR患儿的经胸超声心动图资料,以手术结果为标准,与手术、CTA对比分析其声像图特点。结果 103例CVR构成类型:肺动脉吊带61例,超声心动图诊断准确率96.7%(59/61),漏诊率3.3%(2/61);双主动脉弓21例,超声心动图准确率57.1%(12/21),漏诊率42.9%(9/21);右位主动脉弓伴迷走左锁骨下动脉19例,超声心动图诊断准确率15.8%(3/19),漏诊率84.2%(16/19),左位主动脉弓伴迷走右锁骨下动脉2例,上述血管环畸形CTA全部诊断。103例CVR中伴有心内畸形77例,超声心动图全部诊断,而CTA漏误诊率11.7%(9/77)。结论超声心动图对于诊断肺动脉吊带及心内畸形的灵敏度高,但对双主动脉弓及迷走锁骨下动脉畸形易漏诊;与CTA结合能更好地明确血管环病变的组成及气管形态异常,为手术计划提供完整的信息。     

无名静脉至三血管切面连续扫查诊断胎儿大血管及分支异常的研究

目的:于胎儿大血管和分支异常诊断中无名静脉至三血管切面连续扫查的效果进行探究。方法:对我院2018年1月—2020年1月的60例胎儿大血管及分支异常孕妇做研究,胎儿均做无名静脉至三血管切面连续扫查,总结检查结果。结果:在本课题的60例胎儿中,27例胎儿是血管环,其占比是45.0%,2例胎儿是肺动脉吊带,5例胎儿是主动脉双弓,20例胎儿是迷走锁骨下动脉,其中6例胎儿是右位主动脉弓伴迷走左锁骨下动脉,14例胎儿是左位主动脉弓伴右迷走左锁骨下动脉;15例胎儿是静脉异常,其占比是25.0%,其中,3例胎儿是右上腔静脉缺如伴永存左上腔静脉,4例是下腔静脉离断,8例是主动脉弓下左无名静脉;16例胎儿是主动脉弓内径异常,其占比是26.67%,其中6例胎儿是主动脉弓离断,10例胎儿是主动脉缩窄;2例胎儿是其他异常,其占比是3.33%,其中,1例胎儿是右肺动脉异常起源于主动脉,1例胎儿是主肺动脉窗。结论:通过无名静脉至三血管切面连续扫查,可助于胎儿大血管和分支异常的产前诊断。     

产前超声三血管多切面和主动脉冠状及矢状切面诊断胎儿先天性血管环

目的 观察产前超声三血管多切面和主动脉冠状及矢状切面诊断胎儿先天性血管环的价值。方法 回顾性分析42胎经产前超声诊断的先天性血管环胎儿,于超声三血管多切面、主动脉冠状及矢状切面观察上腔静脉、主动脉弓、肺动脉及动脉导管与气管的位置关系等,并结合产后随访结果分析各切面用于诊断胎儿先天性血管环的价值。结果 42胎中,19胎为右位主动脉弓,其中16胎为右位主动脉弓+迷走左锁骨下动脉+左侧动脉导管,3胎为右位主动脉弓伴镜像分支+左侧动脉导管;17胎为左位主动脉弓伴迷走右锁骨下动脉;3胎为双主动脉弓,其中2胎为右弓优势型、1胎为均衡型;3胎为肺动脉吊带。15胎合并心内畸形。8名孕妇因胎儿染色体异常终止妊娠,9名孕妇于外院分娩而失访;25例新生儿超声心动图或CTA所见均与产前检查结果相符。结论 产前超声三血管多切面结合主动脉冠状及矢状切面有助于检出胎儿先天性血管环。     

超声心动图诊断完全性主动脉弓中断

本文报告一例完全性主动脉弓中断,术前由超声心动图检出,经手术及心血管造影证实。胸骨上切面主动脉根部内径较小(18mm),主动脉瓣三叶,无异常。主动脉弓依次见无名、左颈总、左锁骨下动脉发出,恰于左锁骨下动脉起始端下方主动脉弓形成盲端;多普勒血流信号消失。胸骨旁短轴切面:肺动脉     

主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断的影像学诊断

目的:探讨多种影像技术对主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断的诊断价值。方法:2005—2013年武汉亚洲心脏病医院先后收治7例主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断的患者,对其超声心动图及CT图像、导管检查结果进行回顾性分析。结果:7例患者均行超声心动图及CT检查,诊断为Ⅱ型主-肺动脉间隔缺损(Ho分型法),均合并右侧肺动脉起源于升主动脉及A型主动脉弓离断(Celoria与Patton分型法)。4例患者行导管检查,3例考虑为阻力型肺动脉高压,放弃手术治疗,余4例均行一期手术矫治。结论:超声心动图结合CT检查是诊断本病的可靠手段。手术指征的判断需结合心导管检查、心血管造影。肺动脉压力及阻力是影响手术时机和预后的关键因素。     

产前超声诊断胎儿右位主动脉弓的临床意义

胎儿主动脉弓畸形是指主动脉弓血管走行和(或)血管分支的异常,常见类型有右位主动脉弓伴迷走左锁骨下动脉或无名动脉、镜像型右位主动脉弓、双主动脉弓、围食管后主动脉弓、左位主动脉弓伴迷走右锁骨下动脉[1].右位主动脉弓是指主动脉弓部位于气管和食管的右侧,并跨越右主支气管下行,与降主动脉相连,它是一种常见的先天性主动脉弓畸形,其在成人中发病率为0.1%[2].     

Berry综合征的超声诊断

目的 探讨Berry综合征的超声诊断价值,分析其超声表现类型.方法 回顾性分析6例Berry综合征患者的超声图像,并结合文献分析超声表现的类型.结果 6例患者中,5例超声诊断经手术(3例)或心血管造影(2例)证实,另1例于超声诊断后14 d死亡.综合征畸形包括远端主一肺动脉间隔缺损、右肺动脉起自升主动脉、主动脉弓离断(A型合并动脉导管未闭5例)或狭窄(1例)伴室间隔完整.6例患者根据超声表现可分为两组:A组(3例)表现为间隔近乎缺如,左右肺动脉开口远离,右肺动脉血供主要来自升主动脉,主动脉弓离断;B组(3例)表现为左右肺动脉开口紧邻,联合部骑跨于远端间隔缺损上,右肺动脉血供分别来自升主动脉和主肺动脉,主动脉弓狭窄或离断.结论 经胸超声心动图能准确诊断Berry综合征的各种组合畸形,是术前诊断的首选方法.     

超声心动图在诊断主动脉肺动脉间隔缺损中的应用价值

目的提高超声心动图对主动脉肺动脉间隔缺损（APSD）的诊断准确率，建议临床采用Berry分型。方法11例APSD患者，超声心动图重点扫查大动脉短轴、右室流出道、高位升主动脉长轴、肺动脉干长轴和胸骨上窝主动脉弓等切面，观察主动脉肺动脉间隔是否完整及间隔缺损的大小、部位，肺动脉分支与升主动脉的关系，主动脉弓降段发育状况。结果按Berry分型，11例APSD患者超声诊断分型为：APSDI型6例，Ⅱ。型1例，ⅡB型3例，Ⅲ型1例。2例Ⅱ型APSD患者均合并右肺动脉起源于升主动脉、主动脉弓离断和动脉导管未闭且室间隔完整。术前超声心动图对全部患者的APSD畸形作出了明确诊断，仅漏诊合并动脉导管未闭2例。右心超声造影可明确鉴别APSD与回声中断的假阳性表现。结论超声心动图为无创性诊断APSD的首选方法，其诊断准确性高、重复性强。Berry分型值得向临床推荐。     

产前超声诊断胎儿主动脉弓离断

目的 观察产前超声诊断胎儿主动脉弓离断(IAA)的价值，探讨胎儿IAA的病理演变过程。方法 回顾性分析经产前超声诊断(n=33)或引产后尸检证实(n=6)IAA的39胎胎儿超声心动图表现，并追踪其狭窄段主动脉演变情况。结果 39胎中，32胎产前超声诊断IAA,其中6胎主动脉缩窄(CoA)进行性加重，经超声心动图追踪复查诊断为IAA。其余7胎中，6胎产前超声误诊为主动脉弓病变，后均经尸检证实为IAA;1胎产前超声心动图显示左颈总动脉(LCA)与左锁骨下动脉(LSA)之间主动脉横弓无血流信号而误诊为B型IAA,经引产后尸检证实为LCA与LSA之间主动脉弓严重狭窄但未闭锁。38胎确诊IAA胎儿中，A型18胎、B型19胎、C型1胎；超声心动图表现主要为心脏四腔心切面左心室明显小于右心室或差别不明显，三血管切面主动脉明显小于肺动脉，三血管-气管切面和主动脉弓切面主动脉与降主动脉不相连，主动脉弓切面见主动脉弓弯曲度变小、走行僵直，主动脉、动脉导管及降主动脉之间无正常“V”字形结构；彩色多普勒显示主动脉弓中断处无连续血流信号，动脉导管内见血流反转。6胎最初产前超声心动图表现为CoA的IAA胎儿后经多...     

Isolated Interrupted Aortic Arch: A Case Report and Review of the Literature

An 18-years-old male presented to emergency department after a car accident with the diagnosis of femoral bone fracture. Arterial blood pressure was 160/90 mmHg in both arms. Bilateral femoral and popliteal pulses were extremely weak and there was systolic ejection murmur on the left second intercostals area. Chest X-ray showed rib notching with normal cardiac silhouette. Transthoracic echocardiography showed the aortic interruption just below the left subclavian artery. Aortography showed a complete interruption of the aortic arch (IAA) just distal to the origin of the left subclavian artery. Femoral bone fracture was treated by conservative strategy. A gadolinium contrast-enhanced magnetic resonance angiogram (1.5 T scanners) clearly reaffirmed a complete interruption of the descending aorta, 3.6 cm from the left subclavian artery with extensive collateralizations. Mild degree hypertension was controlled by a long acting calcium channel blocker. Later the patient has been scheduled for elective surgical repair. We aimed to discuss the diagnostic and treatment options of the interrupted aortic arch as being a rare anomaly.     

应用超声心动图对先天性主动脉弓畸形病例的分析

目的应用超声心动图观察分析先天性主动脉弓畸形的类型。方法回顾分析以往病例检查中31例先天性主动脉弓畸形患者的超声心动图表现。所选病例均经手术或心血管造影(CAG)证实。结果31例先天性主动脉弓畸形患者中,右位主动脉弓(RAA)18例(2例伴有椎动脉自主动脉弓发出),主动脉缩窄(COA)5例,主动脉弓部曲折畸形3例,单纯头臂干(无名动脉)发出异常2例,肺动脉单发于主动脉弓2例(1例伴有头臂干发出异常),主动脉弓离断(IAA)1例,其中27例合并其他心内畸形。结论超声心动图对于诊断先天性主动脉弓畸形是一种有效、无创、经济的检查手段,可作为诊断的首选方法。     

胎儿右位主动脉弓的产前超声诊断及分析

目的提高胎儿右位主动脉弓的产前超声诊断正确率。方法分析总结我院诊断的51例右位主动脉弓胎儿产前超声特征。结果 51例右位主动脉弓胎儿中,右位主动脉弓并左锁骨下动脉迷走30例(其中右位主动脉弓并左无名动脉迷走1例),镜面右位主动脉弓21例。单纯右位主动脉弓24例,合并复杂心内外结构畸形24例(镜面右位主动脉弓19例),形成血管环29例。结论不同类型的胎儿右位主动脉弓有不同的结构特点,并有其特殊的产前超声表现,部分镜面右弓和右位主动脉弓并动脉迷走时在产前诊断仍有一定的难度。     

胎儿主动脉弓发育异常产前超声诊断

目的 探讨胎儿主动脉弓发育异常的产前超声诊断价值。方法 回顾性分析45例胎儿主动脉弓发育异常产前超声图像特征。结果 经产后证实胎儿主动脉弓发育异常45例, 其中主动脉弓缩窄15例(13例合并心内畸形,6例合并心外畸形);主动脉弓离断3例(均合并心内畸形);右位主动脉弓并迷走左锁骨下动脉、左位动脉导管12例(1例合并心内畸形);镜面右位主动脉弓14例(7例合并心内畸形,4例合并心外畸形);双主动脉弓1例(未合并心内畸形)。结论 主动脉弓离断、主动脉弓缩窄、镜面右位主动脉弓常合并心内畸形,部分合并心外畸形,产前明确主动脉弓发育异常及其合并心内、心外畸形情况,对于评价胎儿预后有重要指导意义。     

胎儿右位主动脉弓的产前超声诊断

目的评价产前超声诊断胎儿右位主动脉弓的临床价值。方法回顾分析我院自2007年1月至2008年6月期间,产前超声诊断的7例胎儿右侧主动脉弓病例。结果产前超声诊断7例右主动脉弓病例。4例伴有左锁骨下动脉迷走,其中有一例产后诊断合并房间隔缺损,一例合并房间隔缺损及室间隔缺损。结论右主动脉弓可以在胎儿心超的三血管气管观上做出诊断。右主动脉弓可以单独发生,也可以合并其他心脏或心外畸形发生。     

胎儿主动脉弓异常的超声诊断线索和方法研究

目的 探讨胎儿主动脉弓异常的超声诊断方法技巧、诊断线索,提高主动脉弓异常的产前超声诊断率.方法 选择2006年1月至2009年12月在我院行产前系统超声检查并获得结果验证的1472例正常和异常胎儿为研究对象,每个胎儿均进行四腔心切面、左右心室流出道切面、三血管气管切面的观察,当怀疑主动脉弓异常时,进一步获得主动脉弓长轴切面和冠状切面及经过气管的冠状切面.结果 产前超声共诊断148例主动脉弓异常,漏诊1例,92例获得结果验证,包括主动脉弓缩窄28例,主动脉弓离断10例,右位主动脉弓及主动脉弓分支异常52例,双主动脉弓2例.24例合并其他严重心脏畸形.主动脉弓缩窄、主动脉弓离断的线索为三血管气管平面均显示主动脉弓内径和动脉导管内径比例失调,主动脉弓内径异常小,四腔心切面显示左心小,左右心不对称;右位主动脉弓及其分支异常在3VTV平面显示主动脉弓位于气管右侧、主动脉弓和动脉导管之间距离增大呈"U"形和(或)在气管后方有发自降主动脉起始段的血管分支-锁骨下动脉.主动脉弓横切面即三血管气管切面、纵切面、冠状切面的显示率分别为 98.4%、90.0%、81.9%.结论 四腔心切面显示左心小,左右心不对称和3VT平面显示主动脉弓内径异常小,主动脉弓与动脉导管内径比例失调是诊断主动脉弓缩窄、离断的线索,主动脉弓和动脉导管之间距离增大是诊断右位主动脉弓的线索,三血管气管平面是诊断主动脉弓异常最易显示和最敏感的切面,主动脉弓冠状切面、纵切面及经过气管的冠状切面对诊断具有补充和鉴别意义.

Abstract：

Objective To study the ultrasonographic clues and methods for fetal anomalies of the aorta arch and improve prenatal detection of anomalies of the aorta arch.Methods One thousand four hundred and seventy-two cases fetus who were carried out detailed scan and whose results were confirmed were chose as study objects.Every routine fetal echocardiography included four chamber and left and right outflow tract and three-vessel trachea view(3VT).The more views which included longitudinal and coronary view of the aorta arch and coronary view of the trachea and main bronchus were obtained when the abnormality of aorta arch was suspected.Results One hundred and forty-eight cases with anomalies of aorta arch were diagnosed by ultrasonography.One case was misdiagnosed.Ninety-two fetus with anomalies of aorta arch which included 28 aortic coarctation(CoA) and 10 interrupted aortic arch (IAA) and 52 right-side aortic arch and abnormal aortic branch and 2 double aortic arch were confirmed by postmortem or postnatal echocardiography and surgery.Of the 92 confirmed cases,24 had prenatally diagnosed additional complex intracardiac anomalies.All cases with CoA and IAA presented ventricular and/or great arterial disproportion with smaller left ventricle and aorta diameter on four chamber view and 3VT.Right aortic arch (RAA) and abnormal aortic branch(AAB) displayed aortic arch located on the right side of the trachea and increased distance between the aortic arch and arterial duct and abnormal aortic arch branch-subclavian artery originating from the beginning section of the descend aorta which coursed behind the trachea with U-shaped appearance on the 3VT plane.The display rate of the transverse and longitudinal and coronary view of the aorta arch was 98.4%,90.0%,81.9%,respectively.Conclusions Disproportional ventricular and /or great arterial with smaller left ventricle and aorta diameter are the clues for CoA and IAA.Increased distance between the aortic arch and arterial duct is the clue for RSA.The transverse view of the aortic arch 3VT is the most sensitive for detecting the anomalies of the aortic arch and the most easily be obtained.The longitudinal and coronary view of the aorta arch and coronary view of the trachea and main bronchus are helpful in differentiating the anomalies of the aortic arch.     

胎儿主动脉弓异常的产前超声声像图特征

目的 探讨主动脉弓异常的产前超声声像图特征,以提高其产前超声诊断率.方法 回顾性分析73例先天性主动脉弓异常胎儿的产前超声声像图、引产后胎儿尸检结果或新生儿超声心动图结果,并与正常胎儿产前超声图像进行对比,研究主动脉弓异常的产前超声图像特征.结果 73例先天性主动脉弓异常胎儿中,主动脉弓位置异常33例,正常左位主动脉弓伴右锁骨下动脉迷走9例,主动脉弓离断5例,主动脉弓缩窄26例.本组66例经产后证实的病例资料显示主动脉弓异常产前超声诊断准确率为86.36%.最有效的扫查切面是三血管切面和三血管气管切面.结论 先天性主动脉弓异常各类型均有特征性的超声表现,掌握其产前超声诊断的主要切面及产前超声声像图特征,其产前超声诊断准确率较高.但是主动脉弓严重缩窄与主动脉弓离断产前超声鉴别诊断有一定难度.     

Diagnosis of subclavian steal in infants with coarctation of the aorta and interruption of the aortic arch by color-coded Doppler sonography

We report the noninvasive diagnosis of subclavian steal by color-coded Doppler ultrasonography in nine infants with congenital heart disease. The underlying cardiovascular malformations included coarctation of the aorta in four infants, interrupted aortic arch type B in three patients, truncus arteriosus communis type A4 (one patient), and isolation of the right subclavian artery (one patient). In all patients both vertebral arteries could be displayed through the anterior fontanel in coronal sections. Normally the flow in both vertebral arteries, as well as the flow in the basilar artery, is displayed in red. In eight patients with angiographically proved unilateral subclavian steal, an antegrade (red) flow could be shown in one vertebral artery, whereas the contralateral vertebral artery was displayed blue, indicating reverse flow. In one infant with interrupted aortic arch type B and associated aberrant right subclavian artery, both vertebral arteries and the basilar artery were displayed blue, indicating bilateral subclavian steal. Color-coded Doppler sonography seems to be a sensitive, noninvasive method for diagnosing congenital subclavian steal, especially in infants with obstruction of the aortic arch.     

Foreign body sensation: A rare case of dysphagia lusoria in a healthy female

Dysphagia lusoria is a rare disease due to an aberrant right subclavian artery that passes posteriorly between the esophagus and the spine. David Bayford coined the term itself meaning "freak or jest of nature" in 1761 describing a case in which the patient had long term dysphagia that eventually led to death. Most cases of dysphagia are due to an aberrant right subclavian artery running posterior to and causing esophageal compression, but only 20-40% of aberrant arteries actually lead to trachea-esophageal symptoms, including dysphagia. The majority of patients with an aberrant right subclavian artery are asymptomatic. Treatment for dysphagia lusoria varies depending on the severity of the symptoms. Dietary modifications are recommended in patients with mild to moderate symptoms while vascular reconstruction is necessary for patients with severe symptoms. We present a 44-year-old female who was diagnosed with dysphagia lusoria due to a right-sided aortic arch and aberrant left subclavian artery (ALSA) with aneurysmal dilation. Right aortic arch with ALSA is an uncommon arch anomaly, and only occurs in about 0.05% of the population. Prior case reports of dysphagia lusoria differ in that they did not report patients with an uncommon presentation of dysphagia lusoria with aneurysmal dilatation nor was the patient diagnosed in the emergency department.