复杂先天性心脏病合并下腔静脉畸形的彩色多普勒超声诊断

目的：探讨复杂先天性心脏病合并下腔静脉畸形的超声表现,以便作出正确诊断。方法与结果：经彩色多普勒诊断并经心血管造影或手术证实的５例下腔静脉畸形的患者,３例为下腔静脉肾上段中断由奇静脉连接,２例为下腔静脉畸形引流入左房。下腔静脉中断常合并肝静脉的畸形引流。同时,下腔静脉畸形与心房位置异常（心房对称）密切相关。结论：为了正确诊断下腔静脉畸形,应注意对剑下切面的观察,如果短轴切面见主动脉和下腔静脉对称结构消失,长轴切面能同时显示两条大血管,则高度怀疑下腔静脉畸形性。     

复杂先心病合并体静脉畸形的二维及彩色多普勒超声诊断

目的：探讨复杂先天性心脏病合并常见体静脉畸形的超声特征，以便做出正确的诊断。方法：与结果：本文报道经二维和彩色多普勒诊断并经造影或手术证实69例复杂先天性心脏病合并体静脉畸形引流，其中部分患者为多发静脉畸形；69例中合并静脉畸形77例次，包括永存左上腔静脉(LSVC）47例次；左上腔静脉入左房15例次；合并奇静脉异常连接的下腔静脉(IVC)肾上段中断10例次和严重狭窄1例；左无名静脉走行异常4例次。结论：1．心房异构时，体静脉畸形发生率显菩高于心房正位．且异构的性质与体静脉畸形引流类型密切相关；2．为了正确诊断体静脉畸形引流，应进行多切面系统检查，尤其注意对剑下、胸骨上窝和胸骨旁高位切面的观察；3．剑下短轴切面见腹主动脉/下腔静脉与脊柱的对称关系消失，则提示下腔静脉异常；左胸骨旁高位与胸骨上窝切面多能直接显示左上腔静脉，同时左无名静脉非常细小或缺如。如果冠状静脉窦无扩张，则高度怀疑左上腔静脉入左房。左上肢静脉声学造影对左上腔静脉畸形的识别有重要意义。     

磁共振在复杂型先天性心脏病患儿大血管畸形中的诊断价值

【目的】探讨磁共振成像（MRI）扫描技术在复杂型先天性心脏病（CCHD）患儿大血管畸形中的诊断价值。【方法】22例经手术病理证实大血管异常的CCHD患儿,均完成心脏大血管MRI检查,分析其MRI特征。【结果】22例CC HD患儿中经手术病理证实法洛四联症11例,内脏心房异位综合征4例,血管环2例,先天性主动脉缩窄5例,M RI诊断结果与病理结果的符合率为100％。M RI采用多角度断层图像及三维重建图像清晰显示CC HD患儿的心内解剖及其周围大血管根部的解剖和胸腹部脏器的位置与形态。【结论】MRI扫描技术在CCHD患儿大血管异常中诊断价值高,术前正确诊断对先天性心脏病的手术治疗至关重要。     

大血管铸型诊断胎儿异构综合征2例

<正>异构综合征主要表现为复杂心血管系统畸形及内脏位置异常。大血管铸型对复杂系统畸形显示具有优势。现将2例异构综合征病例大血管铸型病理结果介绍如下。病例1孕妇33岁,孕24周,G2P0。孕期无不良接触史,产前遗传学检查结果为唐氏筛查低危。引产后胎儿经大血管铸型显示:右房异构综合征可能:双侧均为右心耳;非均衡型房室间隔缺损;完全性心上型肺静脉异位引流,4条肺静脉共同腔于奇静脉下方汇入右上腔静脉;右室双出口;右位动脉导     

产前超声诊断胎儿异构综合征 12 例分析

目的探讨异构综合征的产前超声表现。方法分析12例异构综合征胎儿的超声资料（部分胎儿引产后经尸检证实）。结果12例均存在共同的内脏异构及复杂的心脏畸形。8例右侧异构综合征主要超声表现为右房同侧异构,下腔静脉与主动脉同侧并行,内脏异构,肺静脉异位引流,房室共同通道,右位主动脉弓,功能性单心室;4例左侧异构综合征主要超声表现为：均存在左房同侧异构,内脏异构,下腔静脉离断,奇静脉异常连接,其中2例主动脉发育不良,1例功能性单心室,1例未表现出心脏其他畸形。结论产前超声可准确诊断异构综合征及其中的心脏复杂畸形。     

经胸超声心动图诊断先天性右位心17例及文献分析

目的：提高经胸超声心动图对先天性右位心的诊断水平。方法：收集超声资料完整并获证实的272例右位心患者(本院17例，文献检索255例)进行综合分析。结果：272例患者中镜像心108例，右旋心133例，左房异构15例，右房异构16例。经胸超声心动图对先天性右位心心房方位诊断的敏感性和特异性均为98．5％，对心室方位诊断的敏感性和特异性均为100％，对心室与大动脉连接诊断的敏感性为99．6％，特异性为94．5％。超声心动图能准确评估相关心内畸形，但对某些大动静脉畸形诊断较困难。结论：经胸超声心动图可作为右位心诊断的首选或筛选方法。     

左房异构及其合并畸形的超声心动图诊断与治疗转归分析

目的分析左房异构及其合并畸形患者的超声心动图诊断特征及分型,并探讨其临床治疗及预后转归。 方法选取2010年1月至2018年10月于首都医科大学附属北京安贞医院行经胸超声心动图检查明确诊断为左房异构的患者47例。对其超声心动图诊断特征及分型、手术治疗方法及转归预后等进行回顾性分析。 结果47例左房异构患者中合并复杂畸形者35例（35/47,74.5%）,其中肺血多型16例,肺血少型19例;合并简单畸形者10例（10/47,21.3%）,无合并心内畸形者2例（2/47,4.2%）。47例左房异构患者中内脏位置异常2例;心脏位置异常14例,其中右位心11例（11/47,23.4%）、中位心3例（3/47,6.4%）;38例合并多脾畸形（38/47,80.9%）,1例合并无脾畸形;42例合并支气管及肺脏畸形（42/47,89.4%）。47例均合并下腔静脉肝段缺如（47/47,100.0%）;合并上腔静脉连接异常者23例（23/47,48.9%）;合并肝静脉连接异常者47例（47/47,100.0%）;合并肺静脉异位引流6例（6/47,12.8%）。47例患者中9例（9/47,19.1%）存在心电图或动态心电图检查结果异常。47例患者中,26例接受矫治或姑息手术治疗,其中21例为复杂畸形,5例为简单畸形。接受手术治疗的26例患者中,4例死亡,均为合并复杂畸形患者。 结论左房异构患者多合并复杂心血管及其他脏器畸形,可通过超声心动图明确诊断。超声心动图对左房异构的确诊及合并畸形的准确评估,对临床治疗方案和目标的制定以及患者预后转归的判定具有重要意义。     

先天性心脏病磁共振成像诊断

作者采用心电图门控磁共振成像技术,检查了33例不同类型先天性心脏病患者,其中紫绀类复杂性先天性心脏病22例。有31例MRI诊断与术后诊断和(或)心血管造影结果相符合,确诊率为93.9％。文中详细介绍了房、室间隔缺损、法乐氏四联症、心脏大血管转位等常见心血管畸形的MRI诊断。认为MRI在先天性心脏病,特别是复杂性先天性心脏病诊断中具有重要的临床价值,当临床诊断发生困难或存在诊断不清楚时,尤其是对那些不适合做心血管造影而又必须明确诊断者,MRI是一种理想的检查方法。     

超声心动图诊断先天性右位心合并复杂畸形的临床价值

目的 观察超声心动图对先天性右位心合并复杂畸形的诊断价值.方法 以手术结果为"金标准",心血管造影结果为对照,应用超声心动图按Van Praagh节段分析法评价62例先天性右位心合并复杂心血管畸形患者的心脏解剖结构.结果 62例右位心患者中镜像心28例,右旋心34例.超声心动图和心血管造影对各节段诊断特异性有高度一致性,两方法相比较无显著性差异(P＞0.05).超声心动图对心房方位、心室形态及房室连接、心室动脉连接及主动脉位置诊断准确性均大于91.3%,但对心房异构及某些动静脉畸形诊断较困难.结论 超声心动图对右位心合并复杂心血管畸形患者大多能作出明确诊断,可作为右位心诊断的首选或筛选方法.     

风湿性心脏病慢性房颤心房扩大与心房电生理的相关性研究

目的：探讨风湿性心脏病慢性房颤左房内径大小对心房电生理特性的影响。方法：选择风湿性心脏病慢性房颤患者16例，术前采用超声心动图测量左心房内径，采用心外膜标测技术测量心房有效不应期（AERP）和波长指数（WLI），并与左房内径行相关性分析。结果：慢性房颤患者左房内径和右房压力明显高于窦性心律患者（P〈0．05），左房内径与AERP和WLI呈负相关（r=-0．882和-0．745）。结论：风心病慢性房颤左心房扩张影响心房的电生理特性，提示左心房的扩张引起心房结构重构，并影响心房的电生理重构而导致房颤的发生和维持。     

右房同形异构及其伴随的心脏畸形的超声心动图研究

目的 探讨右房同形异构及伴随病变的超声心动图诊断方法，以提高对右房同形异构的认识，为制定手术方案提供详细的资料。方法 收集超声心动图资料完整并经证实的10例患者，对其超声心动图顺序节段分析的结果进行分析。结果 本组右房同形异构患者最常见的心脏畸形组合为肺静脉异位引流，左位上腔静脉，完全型房室隔缺损，共同房室瓣，心室大动脉连接异常(包括右型大动脉转位，右室双出口)，肺动脉狭窄或闭锁。未见肺静脉引流正常，肺动脉系统正常及心室—大动脉连接一致；未见左室流出道狭窄及主动脉梗阻。结论 右房同形异构为复杂的疾病综合症，有其特征性心脏伴随病变，超声心动图可为外科治疗提供重要的诊断。     

胎儿左、右侧异构综合征产前超声特征的对比研究

目的 对照病理解剖结果,比较胎儿左侧与右侧异构综合征声像图特征的差异.方法 选择产前超声诊断并经病理解剖证实的胎儿异构综合征病例22例,分析比较左、右侧异构综合征的畸形特征及超声图像的差异.结果 22例胎儿异构综合征中右侧异构18例,左侧异构4例.所有病例均有复杂的心血管畸形及内脏异构.18例右侧异构综合征的主要超声、病理表现为:右房同形异构(17例,94%),内脏异构(17例,94%),肺静脉异位引流(16例,89%),房室共同通道(15例,83%),下腔静脉与主动脉同侧并行(12例,67%),持续性左上腔静脉(11例,61%),右位主动脉弓(10例,56%),功能性单心室(10例,56%).4例左侧异构综合征的超声、病理表现为:全部有左房同形异构、内脏异构及下腔静脉离断,3例并房-室传导阻滞、主动脉发育不良及房室共同通道,2例功能性单心室.结论 内脏异构、房室共同通道、功能性单心室为胎儿左、右侧异构综合征的共同特征,右侧异构综合征多合并肺静脉异位引流、右位主动脉弓、下腔静脉与主动脉同侧并行,左侧异构综合征则以下腔静脉离断、房-室传导阻滞及主动脉发育不良为主要合并声像图表现.

Abstract：

Objective To compare the prenatal ultrasonic characteristics between left and right isomerism as referring to autopsy outcomes.Methods Between November 2007 and July 2010,fetuses with isomerism that were confirmed by autopsy,were identified from 1200 prenatal ultrasonic scans.Their abnormal spectrums and ultrasonic features were analyzed and comparied between left and right isomerism.Results A total of 18 fetuses with right isomerism and 4 fetuses with left isomerism were detected and confirmed.The major findings of the right isomerism were;viscerocardiac heterotaxy (17,94%),anomalous pulmonary venous connection (16,89%),complete atrioventricular septal defect (15,83%),juxtaposition of the descending aorta and inferior vena cava(12,67%),left persistent superior vena cava (11,61%),right aortic arch (10,56%) and univentricle (10,56%).As for the left isomerism,all had viscerocardiac heterotaxy and interruption of inferior vena cave,three of them had heart block,complete atrioventricular septal defect and hypoplasia of aorta,two of them had univentricle.Conclusions Viscerocardiac heterotaxy,complete atrioventricular septal defect,univentricle are the most common anomalies detected.Right isomerism cases usually exist with anomalous pulmonary venous connection,juxtaposition of the descending aorta and inferior vena cava as well as right aortic arch.The left isomerism cases usually have interruption of inferior vena cave,heart block and hypoplasia of aorta.     

Prenatal diagnosis of right atrial isomerism (asplenia-syndrome): case report

Right atrial isomerism (earlier known as asplenia) is a group of malformations including anomalies of the heart and the visceral organs. Complex defects of the heart are normally found, but these are non-specific. The suspected diagnosis can be confirmed most easily by focusing on a specific and segmental examination of the heart. We report on a fetus referred at 22 weeks in whom AV-septal defect was detected. Heart and stomach were on the left side, but the segmental approach allowed the detection of juxtaposed descending aorta and inferior vena cava as typical signs of right isomerism. The suspected malformation could be verified by the additional detection of infra-diaphragmatic total anomalous pulmonary venous drainage by using colour Doppler. Howell-Jolly bodies from fetal blood supported the diagnosis of asplenia. Autopsy after termination of pregnancy demonstrated the typical signs of right isomerism. The paper reviews the typical prenatal findings which lead to the detection of right isomerism in the fetus. Segmental approach should be performed in each fetus with an anomaly of the heart especially in the presence of complex defects.     

Prenatal diagnosis of total anomalous pulmonary venous connection to the portal vein associated with right atrial isomerism.

We report the prenatal diagnosis of total anomalous pulmonary venous connection to the portal system in a 20-week fetus with right atrial isomerism. The apex of the fetal heart pointed to the left, the fetal stomach was on the right, there was a common atrioventricular valve, the left ventricle was small and the abdominal aorta and inferior vena cava were on the left side; all these features were suggestive of right atrial isomerism. An anomalous vein was connected to the portal vein which ascended above the diaphragm and ended in a confluence of pulmonary veins, posterior to the common atrium. Color Doppler imaging helped confirm the diagnosis of total anomalous pulmonary venous connection. The prenatal findings were confirmed on autopsy.     

MSCT和MR三维快速场回波序列诊断儿童双侧气管性支气管

目的 探讨MSCT和MR三维快速场回波（3D-TFE）序列诊断儿童双侧气管性支气管的价值。方法 回顾性分析14例双侧气管性支气管患儿的胸部MSCT或MRI资料。对10例MSCT图像采用最小密度投影技术进行气道重建;对4例采用MR 3D-TFE序列扫描获得气道图像,并以最大密度投影重建气道。根据MSCT或MRI将患儿分为标准型（双侧气管性支气管均从气管隆嵴上方发出）、临界型（双侧气管性支气管均从气管隆嵴起始部发出）和混合型（双侧气管性支气管从不同位置发出）,分析图像特点及合并心脏畸形。结果 MSCT和MR 3D-TFE序列均可清晰显示双侧气管性支气管。14例中,标准型8例（8/14,57.14%）,临界型5例（5/14,35.71%）,混合型1例（1/14,7.14%）。14例（14/14,100%）患儿均合并无脾综合征。较高发的合并心脏畸形包括共同房室通道（12/14,85.71%）、肺动脉狭窄（11/14,78.57%）和永存左上腔静脉（11/14,78.57%）。结论 MSCT和MR 3D-TFE序列对双侧气管性支气管有良好诊断价值。     

Prenatal diagnosis of cardiosplenic syndromes: a 10-year experience.

OBJECTIVE: To assess the accuracy of fetal echocardiography in the prenatal diagnosis of cardiosplenic syndromes and the spectrum of associated anomalies. METHODS: This was a retrospective survey of fetuses in our databases over a period of 10 years with postnatally confirmed prenatal diagnosis of cardiosplenic syndromes. RESULTS: In 32 of 35 fetuses the prenatal diagnosis of cardiosplenic syndromes was confirmed postpartum. Twenty-two fetuses had left isomerism. Their main prenatal ultrasound features were interrupted inferior vena cava (n = 21), complete atrioventricular septal defect (n = 15), viscerocardiac heterotaxy (n = 15), persistent bradyarrhythmia (n = 12) and fetal hydrops or nuchal edema (n = 12). Twelve pregnancies were terminated, two fetuses were stillborn and eight infants survived. Ten fetuses had right isomerism. Their main sonographic features were juxtaposition of the descending aorta and inferior vena cava (n = 7), complete atrioventricular septal defect (n = 7), left persistent superior vena cava (n = 6) and viscerocardiac heterotaxy (n = 6). In this group there was one stillbirth, five infant deaths and four survivors. The overall survival rate and spectrum of other cardiac malformations were similar between the two groups. Prenatal diagnosis of other visceral features of cardiosplenic syndromes was inconsistent. CONCLUSION: Cardiosplenic syndromes can be diagnosed with high accuracy by prenatal sonography. A diagnosis of left isomerism should be strongly suggested in the presence of a combination of at least two of the following: (1) complete atrioventricular septal defect or other structural heart disease; (2) interruption of inferior vena cava with azygos continuation; (3) early fetal heart block; (4) viscerocardiac heterotaxy. Right isomerism should be suspected in the presence of a combination of at least two of the following: (1) structural heart disease, namely complete atrioventricular septal defect; (2) juxtaposition of inferior vena cava and descending aorta; (3) viscerocardiac heterotaxy.     

The syndrome of left isomerism: sonographic findings and outcome in prenatally diagnosed cases.

OBJECTIVE: The purpose of this study was to evaluate the accuracy of the prenatal diagnosis of left isomerism and to assess possible diagnostic and prognostic markers. METHODS: We conducted a retrospective review of all previously unpublished cases of left isomerism diagnosed in the prenatal and postnatal periods in 2 tertiary referral centers in Germany over 15 years. RESULTS: Among 34 fetuses, 31 had a correct prenatal diagnosis of left isomerism; 31 had an interruption of the inferior vena cava with azygos continuation; 22 had different types of viscerocardiac heterotaxy; 13 had heart block; and 28 had cardiac defects, with a high prevalence of atrioventricular septal defects (n = 24), right outflow tract obstruction (n = 11), double-outlet right ventricles (n = 6), and anomalous pulmonary venous return (n = 6). Among the 34 cases, 9 underwent termination of pregnancy; 2 fetuses died in utero; 5 children died in the neonatal period; and 4 children died in infancy. Only the presence of heart block and hydrops was significantly correlated with nonsurvival (P < .05). Fourteen children survived, with a mean follow-up +/- SD of 2.9 +/- 2.6 years. Three survivors underwent single-ventricle palliation, and 1 had successful biventricular repair. Three children were awaiting cardiac repair. The remaining 7 children had minor or no associated cardiac defects and were doing well. CONCLUSIONS: Prenatal diagnosis of left isomerism is feasible, with high accuracy. Important diagnostic pointers are viscerocardiac heterotaxy, complex cardiac malformations, heart block, and interruption of the inferior vena cava. The mortality in fetuses and neonates is high in the presence of heart block and hydrops, whereas the cardiac defects influence the long-term outcome.