胎儿双主动脉弓的超声心动图特征

目的 总结分析胎儿双主动脉弓的超声心动图特征,探讨产前超声心动图诊断胎儿双主动脉弓的应用价值。方法 回顾性分析我院经产前超声心动图诊断为双主动脉弓胎儿的超声心动图及临床资料,分析超声心动图特征,随访产后诊断结果,总结产前超声心动图诊断经验。 结果 共计17例胎儿产前超声心动图诊断为双主动脉弓,其中右弓优势型11例,对称型6例;合并室间隔缺损1例,合并双上腔静脉并冠状静脉增宽2例,无合并其他心内畸形14例;出生6例,终止妊娠2例,9例失访;出生后出现症状2例,无症状4例;接受手术治疗1例。与胎龄相匹配的正常胎儿比较,病例组胎儿的左右心房内径、左右心室内径、卵圆孔大小、升主动脉与肺动脉内径及峰值流速差异均无统计学意义（p＞0.05）。双主动脉弓胎儿典型的超声心动图特征表现为：（1）在三血管-气管（3VT）切面显示两条主动脉,将气管与食管包绕,并与动脉导管形成“9”字形血管环;（2）在3VT切面均显示左位动脉导管;（3）升主动脉冠状切面可见升主动脉发出两条主动脉弓,降主动脉冠状切面可见两条主动脉弓汇入降主动脉;（4）主动脉弓长轴切面可见左、右主动脉弓各发出两个分支;（5）3VT向头侧连续扫查显示两条主动脉弓及分支走行。结论 双主动脉弓胎儿具有独特的产前超声心动图特征,三血管-气管切面对诊断胎儿双主动脉弓具有重要意义,发现左、右两个主动脉弓及分支走行是诊断的关键。     

胎儿双主动脉弓的超声心动图特征分析

目的总结分析胎儿双主动脉弓的超声心动图特征,探讨产前超声心动图对其的诊断价值。方法回顾性分析我院17例经产前超声心动图诊断为双主动脉弓胎儿(病例组)和与其胎龄相匹配的17例正常健康胎儿(对照组)的超声心动图及临床资料,分析双主动脉弓超声心动图特征,并随访产后诊断结果。结果 17例胎儿产前超声心动图诊断为双主动脉弓,其中右弓优势型11例(1例左弓闭锁),对称型6例;合并室间隔缺损1例,合并双上腔静脉并冠状静脉增宽2例,余未合并其他心内畸形。出生6例,终止妊娠2例,失访9例。出生后有临床症状2例,无症状4例;接受手术治疗1例。对照组与病例组胎儿的左右心房内径、左右心室内径、卵圆孔大小、升主动脉与肺动脉内径及峰值流速比较,差异均无统计学意义。双主动脉弓胎儿典型的超声心动图特征表现为:①三血管-气管(3VT)切面显示两条主动脉,将气管与食管包绕,并与动脉导管形成"9"字形或"O"字形血管环;②3VT切面均显示左位动脉导管;③升主动脉冠状切面可见升主动脉发出两条主动脉弓,降主动脉冠状切面可见两条主动脉弓汇入降主动脉;④主动脉弓长轴切面可见左、右主动脉弓各发出两个分支;⑤3VT切面向头侧连续扫查显示两条主动脉弓及分支走行。结论双主动脉弓胎儿具有独特的产前超声心动图特征,3VT切面对诊断胎儿双主动脉弓具有重要意义,发现左、右两个主动脉弓及分支走行是诊断的关键。     

双主动脉弓的产前超声心动图诊断

目的通过分析总结胎儿双主动脉弓(DAA)的超声特征,提高产前超声心动图诊断的准确度。方法回顾性分析产前诊断的6例双主动脉弓胎儿超声心动图图像,总结其超声特征。结果 6例胎儿中右弓优势型4例,左弓优势型1例,均衡型1例。4例胎儿出生后出现相关的临床症状,2例无明显症状;3例行手术治疗。胎儿期超声特征如下:⑴升主动脉长轴切面呈Y字征;(2)在3VT切面显示,主动脉发出左侧及右侧主动脉弓,并将气管和食管包绕其中,形成"O"形血管环;(3)在3VT切面显示动脉导管弓走形于气管及左侧主动脉弓的左侧;(4)在主动脉弓降部冠状切面,二维、彩色多普勒及能量多普勒可显示左侧弓在导管内侧与降主动脉相连,左右主动脉弓分别发出颈总动脉和锁骨下动脉,并最终连接到降主动脉。结论双主动脉弓胎儿期有典型的超声图像,多切面显示左、右主动脉弓及动脉导管弓均与降主动脉连接是诊断的关键;主动脉弓降部冠状切面对该畸形的诊断及鉴别诊断有重要价值。     

胎儿右位主动脉弓产前超声诊断、妊娠结局及漏误诊分析

目的探讨并总结胎儿右位主动脉弓（RAA）超声图像特征、鉴别诊断方法,分析其漏误诊原因,提高胎儿RAA产前超声诊断准确率。 方法选取2014年1月至2017年12月黑龙江省哈尔滨市红十字中心医院31例经产后小儿超声心动图检查、手术或引产后尸体解剖病理证实的胎儿RAA病例,总结胎儿RAA及其合并圆锥动脉干畸形产前超声图像特征、鉴别诊断方法,分析其漏误诊原因并随访其妊娠结局。应用胎儿心脏标准超声切面的灰阶和彩色多普勒血流成像,进行胎儿心脏系统超声检查。当三血管气管切面（3VT）发现主动脉弓位于气管右侧时,再获取气管及其分支冠状切面进一步明确主动脉弓、动脉导管弓与气管的位置关系。 结果产前超声诊断41例胎儿RAA,活产20例,引产15例,失访6例;产后证实31例,产前超声正确诊断25例（25/31,80.65%）,漏误诊6例（6/31,19.35%）。2例左位主动脉弓产前超声均未获取胎儿气管及其分支冠状切面,在3VT切面将支气管横断面误认为气管横断面而误诊为RAA,2例RAA伴左锁骨下动脉迷走（ALSA）左位动脉导管（LDA）,将左颈总动脉误认为发育不良的左弓而误诊为双主动脉弓（DAA）,2例RAA合并心脏圆锥动脉干畸形产前超声漏诊RAA。31例胎儿RAA在3VT切面显示主动脉弓位于气管右侧,不同类型的胎儿RAA在3VT切面形成血管环或无血管环。胎儿RAA合并圆锥动脉干畸形在3VT切面和气管及其分支冠状切面有不同超声表现。20例活产胎儿中13例孤立性RAA、2例合并永存左上腔、1例合并2~3腰椎椎体融合、2例合并室间隔缺损手术治疗、最大年龄随访至3岁半,无呼吸道消化道压迫症状,小儿状态良好。1例IDD型矫正型大动脉转位并RAA,未经手术治疗,目前小儿11个月,状态良好。1例合并食道闭锁产后14 d新生儿死亡。6例行胎儿染色体核型检查,5例染色体核型正常,1例合并心内外严重多发畸形胎儿染色体核型检查为18-三体。 结论3VT切面是诊断胎儿RAA的主要切面,联合应用气管及其分支冠状切面可提高产前超声诊断准确率。单纯性RAA多数预后好、RAA合并严重心内外畸形预后与其合并畸形严重程度有关。     

胎儿主动脉弓离断超声表现1例

孕妇,26岁,孕25周.来我院做产前超声检查.超声显示:胎儿双顶径6.4 cm,胎心率139次/min,脊柱排列规则、连续;腹腔脏器未见异常,颜面部及四肢扫查未见异常.胎儿心脏四腔观示左心室略小,右心室略大,卵圆孔大小约0.52 cm;三尖瓣测及大量反流信号;主动脉、肺动脉起始部呈交叉状;左室流出道切面显示升主动脉明显变细,内径约0.32 cm,升主动脉正常上升弧度消失,主动脉弓切面不能显示正常主动脉弓.头臂动脉从升主动脉远端发出,走形角度异常(图1),肺动脉明显增宽,内径0.72 cm,可显示完整的动脉导管弓(图2),CDFI示升主动脉血流速度164.4 cm/s,肺动脉主干血流速度58.2cm/s.超声提示:①中期妊娠,单活胎;②胎儿心脏发育异常,主动脉狭窄,主动脉弓不能显示.     

胎儿主动脉弓离断的超声诊断及畸形特征分析

目的 研究胎儿主动脉弓离断的产前超声诊断、鉴别诊断技巧及其畸形特征.方法 所有胎儿均常规获得四腔心切面、左右心室流出道切面、三血管气管切面(3VT)的灰阶和彩色多普勒血流显像.当发现左心和主动脉内径异常小,左/右心室比例和/或升主动脉内径/肺动脉内径比例失调怀疑主动脉弓异常时,进一步获得主动脉弓纵切面和冠状切面明确诊断.结果 产前超声共诊断12例主动脉弓离断,均表现为升主动脉内径相对细小,升主动脉/肺动脉内径比例失调;均合并室间隔缺损.通过解剖证实4例为Bb型主动脉弓离断合并胸腺缺如,2例为A型主动脉弓离断,3例为严重主动脉弓缩窄,3例失访.结论 主动脉内径相对细小,升主动脉内径/肺动脉内径比例失调及室间隔缺损等声像特征是产前诊断主动脉弓离断的重要线索,严重主动脉弓缩窄易误诊为主动脉弓离断,Bb型主动脉弓离断常合并胸腺缺如.     

3VT切面上主动脉闭锁及肺动脉闭锁的超声诊断与鉴别诊断价值

目的:研究分析胎儿主动脉闭锁和肺动脉闭锁超声诊断使用3VT切面的价值,为临床诊断提供参考。方法:为6例主动脉闭锁和9例肺动脉闭锁病例使用3VT切面超声诊断,根据超声图情况来进行判断分析特点,对其临床价值进行分析。结果:主动脉闭锁的6例患者,可以观察到主动脉管腔变小,肺动脉管腔变大,经彩超诊断后,主动脉和左心室没有血流联通。使用3VT切面超声检查显示动脉导管弓、主动脉弓、上腔静脉三条血管的大小比例不平衡,主动脉弓有逆向血流情况存在,肺动脉为正向血流。肺动脉闭锁的9例患者有室间隔缺损情况,有右向左分流信号。肺主动脉的起始段模糊,有细小回声,主动脉壁和室间隔的回声间断,主动脉在缺损间隔上,彩超显示肺动脉和右心室没有血流联通。肺动脉闭锁患者的3VT切面图显示主动脉管径增加,肺动脉管径变小,动脉导管弓、主动脉弓、上腔静脉三条血管比例消失,彩超为肺动脉无前向血流,主动脉无逆向血流。结论:胎儿心脏大血管畸形可以使用3VT切面超声检查进行诊断,对主动脉闭锁、肺动脉闭锁进行超声诊断的价值非常高,实际使用的时候,要对系统的安全性,全面性进行观察分析,提供正确的诊断。     

主动脉弓缩窄的产前超声诊断

目的探讨胎儿主动脉弓缩窄(CoA)的产前超声诊断线索、诊断方法与技巧及畸形特征。方法所有胎儿均常规获得四腔心切面、左右心室流出道切面、三血管气管(3VT)切面(即主动脉弓横切面)的灰阶和彩色多普勒血流显像。当四腔心切面发现左心系统偏小、3VT切面显示主动脉弓和动脉导管内径比例失调疑CoA时,进一步获得主动脉弓纵切面和冠状切面(Y平面)并测量峡部内径。结果产前超声共诊断56例CoA患儿,28例有解剖或新生儿超声心动图和手术结果,产前超声误诊和漏诊4例。其中8例合并单心室、心内膜垫缺损、右心室双出口、大动脉转位、永存动脉干等其他严重复杂心脏畸形,7例合并心脏外严重畸形。22例合并室间隔缺损(VSD)和永存左上腔静脉(LSVC)等非复杂心脏畸形。产前超声诊断的28例CoA患儿均首先于3VT切面发现两大动脉内径比例失调,主动脉弓异常小,四腔心切面显示左、右心室比例不对称,左心明显小于右心。此28例CoA患儿中有23例(82.1%)同时获得了3VT切面、主动脉弓纵切面及Y平面,14例于主动脉弓纵切面显示有来自降主动脉的反流。而误诊和漏诊的4例CoA患儿均未能获得满意的主动脉弓纵切面和Y平面。结论 3VT切面显示主动脉弓和动脉导管内径比例失调和四腔心切面不对称、左心偏小是CoA的诊断线索,进一步获取主动脉弓的特殊切面以获得至少2个平面的印证是提高CoA产前诊断率的主要技巧。CoA可以单独发生,也可合并其他心内外畸形。     

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...     

超声心动图多切面对胎儿右位主动脉弓分型诊断及其与双主动脉弓鉴别的价值

目的：探讨超声心动图在胎儿右位主动脉弓（RAA）分型及其与双主动脉弓（DAA）鉴别诊断中的价值。方法：选择2014年1月至2017年12月经中孕期常规超声筛查初次诊断为RAA和DAA的胎儿55例,联合应用超声心动图三血管气管切面和主动脉弓降部冠状切面再次加以诊断,并随访至出生。比较初次筛查和再次诊断的差异,以及这2种心脏畸形在超声心动图不同切面的特征。结果：初次超声筛查出RAA组46例、DAA组9例。其中,RAA组经联合应用三血管气管切面和主动脉弓降部冠状切面进一步明确分型,发现RAA伴迷走左锁骨下动脉28例、伴镜像分支18例;出生后完成随访39例,均确诊,且无气管和食管压迫症状,无须行手术纠治。DAA组经联合多切面扫查,发现4例在初次筛查时误诊,实为RAA伴镜像分支,误诊率达44.4%,除1例失访外,其余3例均于出生后确诊;另5例确诊DAA胎儿中,有1例引产,其余出生后均表现明显的气管压迫症状,其中3例顺利完成血管环纠治手术且预后良好。结论：联合应用超声心动图三血管气管切面和主动脉弓降部冠状切面能有效鉴别RAA与DAA。     

主动脉弓降部异常的超声诊断

目的　提高超声对主动脉弓中断 (IAA)、主动脉缩窄 (CoA)、右位主动脉弓 (RAA )三类主动脉弓降部异常的诊断能力。方法　回顾性分析 5 2例主动脉弓降部异常患者的胸前及胸骨上窝二维及多普勒超声表现。结果　IAA表现为主动脉弓部成为盲端 ,降主动脉与肺动脉相连 ,最常合并室间隔缺损 ,均有重度肺高压等。CoA表现为主动脉峡部狭窄 ,降主动脉常窄后扩张 ,腹主动脉内血流频谱异常等 ,部分无心内畸形 ,可通过胸骨上窝及心内表现不同与IAA加以鉴别。RAA表现为主动脉弓弯向右侧 ,标准切面观察时与IAA相似。大部分合并复杂畸形。结论　超声心动图对于诊断主动脉弓降部异常是一种有效、无创、经济的检查手段。胸骨上窝切面超声表现是诊断本病的关键 ,有必要作为常规检查切面     

Fetal sonographic diagnosis of aortic arch anomalies.

Aortic arch anomalies refer to congenital abnormalities of the position or branching pattern, or both of the aortic arch. Although aortic arch anomalies are not uncommon, reports on their prenatal diagnosis are scarce. Insight into the hypothetical arch model is crucial to understanding anomalies of the aortic arch in the fetus. Recognition of the trachea, three major vessels, ductus arteriosus and descending aorta in the axial views of the upper mediastinum is necessary for a complete fetal cardiac assessment. Clues to aortic arch anomalies include abnormal position of the descending aorta, absence of the normal 'V'-shaped confluence of the ductal and aortic arches, a gap between the ascending aorta and main pulmonary artery in the three-vessel view, and an abnormal vessel behind the trachea with or without a vascular loop or ring around the trachea. Meticulous attention to anatomic landmarks will lead to successful prenatal diagnosis of important vascular rings making early postnatal management possible.     

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.     

应用超声三血管气管切面诊断胎儿主动脉弓异常

  目的  研究超声三血管气管切面对于胎儿主动脉弓异常的诊断价值。  方法  回顾性分析2010年5月至2014年5月北京协和医院诊断的17例胎儿主动脉弓异常的产前超声图像资料, 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的产前超声特征, 重点关注三血管气管切面的表现。  结果  17例主动脉弓异常在三血管气管切面均有阳性表现。其中主动脉弓缩窄3例, 表现为主动脉弓细小; 主动脉弓离断7例, 表现为主动脉变细, 不能连接至降主动脉; 右位主动脉弓合并迷走左锁骨下动脉5例, 可见环绕气管的"U"形血管环; 右位主动脉弓合并镜像分支2例, 可见主动脉弓位于气管右侧, 动脉导管不汇入降主动脉。  结论  三血管气管切面是提示胎儿主动脉弓异常敏感且有效的切面, 中孕超声筛查时应重视该切面, 早期发现异常并给予适当的遗传咨询。     

Right aortic arch detected in fetal life.

OBJECTIVE: To evaluate the prenatal distribution, associated conditions and outcome of the different types of right aortic arch (RAA) detected in fetal life. METHODS: This was a retrospective review of all cases of RAA detected prenatally between 1998 and 2005 in two tertiary referral centers. RESULTS: In the study period 71 cases of RAA were detected; 26 (37%) had RAA with aberrant left subclavian artery, 23 (32%) had RAA with mirror-image branching, 20 (28%) had RAA of unknown type and two (3%) had double aortic arch. While 20/26 cases with RAA and aberrant left subclavian artery were isolated findings, all 23 cases with RAA and mirror-image branching were associated with cardiac defects, namely tetralogy of Fallot (43%) or pulmonary atresia with ventricular septal defect (22%). Of the 20 cases with RAA, 19 of unknown type were associated with heterotaxy syndromes and had additional cardiac malformations and ambiguities of the situs. The two cases with DAA were isolated findings. Seven cases in our series (10%) had a microdeletion 22q11 and these were significantly associated with extracardiac malformations. The outcome in our series depended solely on the associated cardiac and extracardiac malformations, with the exception of one infant with isolated DAA, in whom a surgical correction was warranted. CONCLUSIONS: RAA detected in fetal life is associated frequently with other cardiac/non-cardiac malformations, heterotaxy syndromes and microdeletions 22q11. The associated conditions vary depending on the branching type of the brachiocephalic vessels and the presence of extracardiac malformations.     

Prenatal detection of ductal-dependent congenital heart disease: how can things be made easier?

OBJECTIVE: To improve the detection of ductal dependence in fetuses with severe anomalies of the outflow tracts by observing, with directional power Doppler, reverse flow through the aortic arch or ductus arteriosus in a transverse view of the upper mediastinum. METHODS: A slight cranial move of the ultrasound beam from the three-vessel view allows the transverse view of the aortic arch and ductus arteriosus to be visualized simultaneously. This view is orthogonal to the fetal body axis and parallel to the plane of the four-chamber view. In normal fetuses, directional power Doppler interrogation at this level identifies forward flow in both oblique vessels. RESULTS: We examined 43 fetuses with cardiac defects. In five of the cases, there was reversed flow in the aortic arch or ductus arteriosus in addition to severe anomalies of the outflow tracts, including four with hypoplastic left ventricle and one with pulmonary atresia. CONCLUSIONS: Prenatal detection of reversed flow in the aortic arch or ductus arteriosus is associated with complex congenital heart disease with major diminution of forward flow to the corresponding great vessels.     

主动脉弓中断的超声心动图诊断

目的探讨主动脉弓中断的超声探测方法及其超声特征表现.方法 8例主动脉弓中断患者,每例均于剑突下、心尖、胸骨旁及胸骨上窝等透声窗全面扫查,按顺序分段诊断分析.结果超声心动图确诊8例主动脉弓中断,准确率100%.其特征性超声表现为胸骨上窝切面主动脉弓降部显示困难,弓部曲线多较直、较长;弓部与降主动脉失去明显联系;弓部为盲端,血流中断,降主动脉与肺动脉相连,常合并室间隔缺损,动脉导管未闭,均有重度肺动脉高压等.结论超声可明确诊断主动脉弓离断;常规胸骨上窝探查,注意探测方法,可提高检出率和诊断率.     

Isolated interrupted aortic arch in an adult: A case report

BACKGROUNDInterrupted aortic arch (IAA) is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries. It is usually found within a few hours or days of birth. Without surgery, the chances of survival are low. If IAA patients have an effective collateral circulation established, they can survive into adulthood. However, IAA in adults is extremely rare, with few reported cases.CASE SUMMARYA 27-year-old woman presented with a 6-year history of progressively worsening shortness of breath and chest tightness on exertion. She had cyanotic lips and clubbing of the fingers. A transthoracic echocardiogram revealed an enlarged heart and dilation of the main pulmonary artery. There was an abnormal 9 mm passage between the descending aorta and pulmonary artery. The ventricular septal outflow tract had a 14 mm defect. Doppler ultrasound suggested a patent ductus arteriosus and computed tomographic angiography showed the absence of the aortic arch. The diagnoses were ventricular septal defect, patent ductus arteriosus, and definite interruption of the aortic arch. Although surgical correction was recommended, the patient declined due to the surgical risks and was treated with medications to reduce pulmonary artery pressure and treat heart failure. Her condition has been stable for 12 mo of follow-up.CONCLUSIONAlthough rare, IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.     

Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis.

OBJECTIVE: To describe a novel, sonographic approach for in-utero evaluation of normal and abnormal aortic arch. METHODS: Aortic arch was evaluated by imaging of the axial view of the upper fetal mediastinum. The normal left aortic arch was defined by the V-shaped appearance of the junction between the ductus arteriosus and aortic arch, with the trachea situated posteriorly. Right and double aortic arches were diagnosed when the great vessels appeared U-shaped, with intermediate location of the trachea. RESULTS: Between 1997 and 1999, 18 347 women were scanned in three prenatal centers, and pathological findings were prospectively recorded. In a retrospective analysis of the records, we identified 19 fetuses (0.1%) with atypical, U-shaped appearance, and no other structural abnormalities present. With the exception of one fetus with a ventricular septal defect, no congenital cardiac defects were present. Right aortic arch was found in 18 cases, while color Doppler made it possible to diagnose one case with double aortic arch, and one fetus was demonstrated as having Kommerell's diverticulum. In all 18 cases, a left descending aorta and left ductus arteriosus were present, the latter coursing to the left of the trachea, forming a loose partial vascular ring. All were asymptomatic at birth and early infancy. The fetus with double aortic arch that had a true vascular ring underwent early infantile correction. CONCLUSIONS: It is possible to diagnose right and double fetal aortic arch using prenatal ultrasound. The use of color Doppler facilitated in-utero evaluation of possible complications, such as true vascular ring.